Report on the Terminology for Female Pelvic Floor 1820 Dysfunction

2 AN INTERNATIONAL CONTINENCE SOCIETY (ICS) REPORT 3 ON THE TERMINOLOGY FOR FEMALE PELVIC FLOOR FISTULAS – IN ASSOCIATION WITH THE

4 AMERICAN UROGYNECOLOGICAL SOCIETY – VERSION 17 5 6 Soheir Elneil#, Lauri Romanzi#, Judith Goh#, Bernard Haylen#*, Grace Chen, 7 Gamal Ghoniem, Munir’deen Ijaiya, Soo Kwon, Joseph Lee, Sherif Mourad, Rajeev Ramanah, 8 Mohan Regmi, Raheela Mohsin Rivzi, Rebecca Rogers, Jonothan Sharp, Vivian Sung, 9 # SE, LR and JG are equal first authors (Production & Content); also #BH (Mentor, Review, Production) 10 11 Standardization Steering Committees ICS*, Joint ICS - AUGS Working Group on the 12 Terminology for Pelvic Floor Fistula 13 14 Sohier Elneil: University College London Hospitals. U.K. [email protected] 15 Lauri Romanzi: Harvard Medical School. New York, NY, USA [email protected] 16 Judith Goh: Griffith University, Queensland. Australia. [email protected] 17 Bernard Haylen: University of New South Wales, Sydney, Australia. [email protected] 18 Chi Chiung Grace Chen: John Hopkins University, Baltimore, Maryland. U.S.A. [email protected] 19 Gamal Ghoniem: UC Irvine Health, Irvine. California. U.S.A. [email protected] 20 Munir’deen Ijaiya: University of IIorin, Kwara State, Nigeria. [email protected] 21 Soo Kwon: Zucker School of Medicine, New York. NY. U.S.A. [email protected] 22 Joseph Lee University of New South Wales, Sydney, Australia. [email protected] 23 Sherif Mourad: Ain Shams University, Cairo. Egypt. [email protected] 24 Rajeev Ramanah: CHU Besancon, Besancon. France. [email protected] 25 Mohan Regmi: BP Koirala Institute, Dharan. Nepal. [email protected] 26 Raheela Mohsin Rizvi: Aga Khan University, Karachi. Pakistan [email protected] 27 Rebecca Rogers: University of Texas, Austin. Texas. U.S.A [email protected] 28 Jonothan Shaw: Fenwek Hospital, Bomet. Kenya. [email protected] 29 Vivian Sung: Woman & Infants Hospital. Rhode Island. U.S.A. [email protected] 30 31 Correspondence to: 32 Professor Judith Goh 33 Greenslopes Private Hospital 34 Greenslopes. 4120. Queensland. Australia. 35 Phone: +61 7 3847 9909; FAX : +61 7 3847 6433 Email: [email protected]

36 ABSTRACT 37 38 Introduction: The terminology for female pelvic floor fistula (PFF) needs to be defined and organized 39 in a clinically based consensus Report. 40 41 Methods: This Report combines the input of members of the International Continence Society (ICS) 42 in association with the American Urogynecological Society (AUGS), assisted at intervals by external 43 referees. Appropriate core clinical categories and a sub-classification were developed to give a coding 44 to definitions. An extensive process of 16 rounds of internal and external review was involved to 45 examine each definition, with decision-making by collective opinion (consensus). 46 47 Results: A Terminology Report for female PFF or genital tract fistula (GTF), encompassing 416 (188 48 NEW) separate definitions, has been developed. It is clinically based with the most common 49 diagnoses defined. Clarity and user-friendliness have been key aims to make it interpretable by 50 practitioners and trainees in different specialty groups involved in female pelvic floor dysfunction 51 and PFF. Female-specific imaging (ultrasound, radiology and MRI) and conservative and surgical 52 managements as well as appropriate figures have been included to supplement and clarify the text. 53 Interval (5-10 year) review is anticipated to keep the document updated and as widely acceptable as 54 possible. 55 56 Conclusion: A consensus-based Terminology Report for female PFF has been produced to aid clinical 57 practice and research. 58 59 60 61 62 63 64 65 66 67

68 DISCLOSURES: 69 70 Sohier Elneil: Saluda Medical, Astellas, Bluewind Medical. Author and Editor of Chapters, Manuals 71 and Papers on Fistula which do not conflict with the contents of this document

72 Lauri Romanzi: No disclosures. 73 74 Judith Goh: No disclosures. 75 Bernard Haylen: No disclosures. 76 Chi Chiung Grace Chen: No disclosures. 77 Gamal Ghoniem: Cogentix Medical, Laborie 78 Munir’deen Aderemi Ijaiya: No disclosures 79 Soo Kwon: No disclosures 80 Joseph Lee: Astellas. BSCI 81 Sherif Mourad: Astellas, Ferring 82 Rajeev Ramanah: No disclosures 83 Mohan Regmi: No disclosures 84 Raheela Mohsin Rivzi: No disclosures 85 Rebecca Rogers: Astellas, royalties from Uptodate, Travel and stipend from IUGA, ACOG and ABOG 86 Jonothan Sharp: No disclosure 87 Vivian Sung: No disclosures 88 89 90 WORDS: ABSTRACT: 204 words; TEXT: Introduction to Section 9: 13,383 words 91 FIGURES: 35

92 INTRODUCTION:

93 94 Fistula (Latin: fistula – ‘pipe, flute’) refers to an abnormal or surgically made connection 95 between a hollow or tubular organ and the body surface, or between two hollow or tubular 96 organs. The plural noun may be either fistulas or fistulae - fistulas will be used. 97 98 Pelvic Floor Fistula (PFF) refers to a fistula affecting the upper or lower genital tract including 99 the uterus, cervix, vagina and/or the different vaginal compartments and the neighbouring 100 organs such as upper and lower urinary tract (ureter, bladder, urethra) and lower bowel 101 (distal colon, rectum, anus). The term Genital Tract fistula (GTF) should not be used. A 102 diagnosis of PFF fits the established model of symptoms corroborated by clear clinical signs 103 and commensurate evaluation test results, starting with a woman having urinary or fecal 104 incontinence symptoms, usually per vagina. 105 106 There is currently no single document encompassing all elements required for diagnoses in 107 female PFF that includes a full outline of the terminology for symptoms, clinical examination 108 signs, and diagnostic investigations. It would also encompass aetiology, classification, and 109 terminology for the different non-surgical and surgical treatment modalities. 110 111 Core terminology documents that will be referenced are: (i) 2010 IUGA-ICS Joint Terminology 112 Report on Female Pelvic Floor Dysfunction1 and (ii) the equivalent 2019 Male Terminology for 113 Lower Urinary Tract and Pelvic Floor Dysfunction (with its greatly expanded range of 114 definitions)2. Also referenced will be the 2016 IUGA-ICS Joint Terminology Report on Pelvic 115 Organ Prolapse3 and the World Health Organization’s fistula publication4,5. An original aim of 116 the IUGA-ICS Joint Terminology reports1,2 has been to provide a general terminology, forming 117 the “core” terminology to which more specific terminologies can be attached. Reference will 118 also be made to three other published Standardization Reports6-8 and 6 joint IUGA-ICS Female 119 Terminology Reports9-14. 120 121 No standardization document exists on female PFF, though work by groups in the field 122 including the International Society of Obstetric Fistula Surgeons, the World Health 123 Organization International Classification of Disease System, and the International Obstetric 3

124 Fistula Working Group at the United Nations Population Fund (UNFPA) have defined 125 segments of PFF terminology that were reviewed pursuant to the creation of this 126 document15. To devise this first PFF standardization document, the PFF Working Group 127 reviewed all available published documents that used a clinical framework to develop 128 terminology incorporating fistula aetiologies, symptoms, signs, staging and classifications, 129 investigations, diagnoses, and treatments. By including concurrent and subsequent pelvic 130 floor disorders, this document functions as a patient-centred terminology resource that

131 reflects frameworks for cost-effective service integration16-17. 132 133 Female-specific imaging advances in urodynamics, video-endoscopic images, ultrasound, 134 radiology and MRI have been commonly used by surgeons in well-resourced settings and are 135 increasingly available to surgeons in resource-constrained settings across sub-Saharan Africa 136 and South and South East Asia. The indications for imaging in PFF and the utility of multi- 137 channel urodynamics (UDS) in the evaluation and management of women with lower urinary 138 tract symptoms (LUTS) after successful urinary tract fistula closure or LUTS concurrent with

139 rectovaginal fistula will be illustrated in this terminology document. 140 141 The terminology document defines methods for non-surgical treatment of fistula with 142 catheter, debridement and fulguration. This report acknowledges that PFF may not occur in 143 isolation but may be associated with pelvic organ prolapse (POP)18-21 and voiding, defecatory 144 and/or sexual dysfunctions and/or other pelvic floor dysfunction, and/or other diagnoses of 145 musculo-skeletal, renal, reproductive, and mental health aetiologies. 146 147 As with all ICS Terminology documents, this terminology report collates the definitions of PFF 148 terms, i.e. “the technical or special terms or expressions used in a business, art science or 149 special subject” or “nomenclature in a field of study”22. Emphasis will continue the ICS 150 tradition of terms in current use in the relevant peer-reviewed literature. The aim is to assist 151 clinical practice and research. Some new and revised terms have been included. Explanatory 152 notes on definitions have been referred, where possible, to the “Footnotes” section. 153 154 This document aims to comprehensively cover all terminology for PFF management: (i) for 155 any aetiology (including congenital, obstetric and iatrogenic); (ii) for management anywhere

156 in the world (though we realize there will be vast differences in access to investigations and 157 other resources); (iii) inclusive of intercurrent pathology (e.g. pelvic organ prolapse); (iv) 158 inclusive of the latest update of ICS terminology on lower urinary tract dysfunctions2, (so 159 there is no need for the reader to seek additional documents). It’s all included in the current 160 document. 161 162 Like all the other joint ICS female-specific terminology reports, every effort has been made to 163 ensure this Report is: 164 165 (1) User-friendly: It should be able to be understood by all clinical and research users. 166 167 (2) Clinically based: Symptoms, signs and validated assessments/investigations should be 168 presented for use in forming workable diagnoses for PFF and associated dysfunctions. 169 Sections 1-6 will address aetiology, classification, symptoms, signs, and investigations and 170 imaging for PFF and associated diagnoses. Radiologic investigations including Magnetic 171 Resonance Imaging (MRI) and Computerized Tomography (CT) have also been incorporated. 172 Section 7 will address fistula diagnoses, possible fistula-related diagnoses and common co- 173 morbidity diagnoses. Sections 8 and 9 will list the terminology for conservative and surgical 174 treatments for PFF. 175 176 (3) Origin: Where a term’s existing definition (from one of multiple sources used) is deemed 177 appropriate, that definition will be included and duly referenced. Many terms in female pelvic 178 floor prolapse and dysfunction, because of their long-term use, have now become generic, as 179 apparent by their listing in medical dictionaries. The terms used in PFF will be defined for the 180 first time in this document. 181 182 (4) Able to provide explanations: Where a specific explanation is deemed appropriate to 183 describe a change from earlier definitions or to qualify the current definition, this will be 184 included as an addendum to this paper (Footnote [FN] 1, 2, 3….). Wherever possible, 185 evidence-based medical principles will be followed.

186 Table 1: Total, New and Changed definitions (compared with previous definitions in the ICS 187 Glossary).

Section New Changed Total Definitions/Descriptors Definitions/ Descriptors Introduction, 16 0 16 Aetiology Classification 27 0 27 Symptoms 22 0 90 Signs 45 3 56 Investigations 11 0 71 Imaging 4 0 31 Diagnoses 37 0 64 Conservative 10 1 29 Management Surgical 16 0 32 Management Total 188 (45%) 4 (1%) 416 188 189 It is suggested that acknowledgement of these standards in written publications related to 190 female PFF, be indicated by a footnote to the section “Methods and Materials” or its 191 equivalent, to read as follows: “Methods, definitions and units conform to the standards 192 jointly recommended by the International Continence Society, except where specifically 193 noted”. 194

195 SECTION 1: AETIOLOGY 196 The aetiology of a PFF can be many and varied, including both congenital and acquired causes. 197 To further clarify aetiology as currently used within the academic fistula surgeon community 198 of practice, aetiologies are further stratified into two groups based on whether or not the 199 fistula is related to childbirth, or not related to childbirth. Congenital causes define aetiology 200 across the urinary, genital and anorectal tracts. Acquired causes include obstetric, iatrogenic, 201 mixed obstetric-iatrogenic, traumatic, inflammatory, infection-based and fistula caused by 202 cancer. 203 204 1.1 Childbirth related 205 1.1.1 Obstetric fistula (OF): Due to prolonged obstructed labour with a fistula from 206 the urinary tract and/or ano-rectal tract to the genital tract caused by ischemia and 207 necrosis. NEW 208 1.1.2 Iatrogenic childbirth-related fistula (ICRF): Fistula is directly due to injury to 209 urinary tract/ano-rectal area during operative delivery (caesarean section/caesarean 210 hysterectomy or instrumental delivery including episiotomy). NEW 211 1.1.3 Mixed obstetric and iatrogenic fistula (MOIF): Fistula related to operative 212 delivery for prolonged obstructed labour. NEW 213 1.1.3.1 Tissue integrity already compromised by obstructed labour prior to 214 operative delivery. NEW 215 216 1.2 Non-childbirth related 217 1.2.1 Congenital fistula (ConF): Fistula present from birth. NEW 218 1.2.1.1 Hypospadias: opening of the urethra other than at the site of external 219 urinary meatus. e.g. low- or mid- vaginal. NEW 220 1.2.1.2 Ectopic ureter: ureter terminating at a site other than the bladder. 221 NEW

222 1.2.1.3 Total perineal defect of genital tract FN 1.1 absent perineal body NEW 223 1.2.1.4 Imperforate anus with spontaneous rectovaginal rupture of anorectal 224 tract: Rectovaginal fistula caused by pressure in the rectum due to an imperforate 225 anus. NEW

226 1.2.2 Iatrogenic fistula (IF): Pelvic floor fistula occurring after non-obstetric pelvic 227 procedures/surgery. NEW 228 1.2.3 Traumatic fistula (TF): Fistula due to trauma to the genital tract such as pelvic 229 crush/impalement injury, sexual violence, female genital tract cutting, insertion of 230 vaginal foreign materials (packing with herbs/stones/salt/foreign bodies). NEW 231 1.2.4 Inflammatory fistula (InF): Fistula due to inflammatory conditions such as 232 inflammatory bowel disease (e.g. Crohns, ulcerative colitis). NEW 233 1.2.5 Infection-related fistula (IxF): Fistula due to infections/abscess (e.g. 234 tuberculosis, schistosomiasis, infectious breakdowns of obstetric perineal trauma, 235 perianal abscess). NEW 236 1.2.6 Cancer-related fistula (CF): Fistula due to tissue compromise from malignancy 237 or from treatment of malignancy such as radiation therapy or surgery. NEW 238 239 Footnotes for section 1 240 FN 1.1: Total perineal defect: see section 2 241

242 SECTION 2: CLASSIFICATION

243 No consensus on a classification system for female pelvic floor fistula exists23 (current 244 proposed classification systems are outlined in Section 2 footnotes). Terms outlined below 245 will denote the proximal/distal locations along the urinary, colorectal and genital tracts and 246 site-specific categories (e.g. urethro-vaginal fistula). Fistulas may, however be large, straddle 247 both proximal/distal locations and involve more than one anatomical site. More than one 248 fistula may be present. The amount of scarring and residual tissue present (for surgical 249 purposes) will be variable. The fistula may also be described by its anatomical location and 250 antecedent event (e.g. obstetric, iatrogenic, combined). 251 252 2.1 BASIC CATEGORIES OF PELVIC FLOOR FISTULA 253 The following terms are defined, each in relation to the hollow organ system component 254 involved in the fistula defect (Fig 1). These are localizing/descriptive terms and not a 255 classification system as such. The following acronyms will be used: F (fistula);

256 V (bladder/vesico); Va (vaginal); U (uterine); Cx (cervical); Ur (ureteric); R (rectal); Co (colon); 257 Pe (perineal); AC (ano-cutaneous). 258 2.1.1 Urethro-vaginal fistula (UVaF): abnormal connection between the urethra and the 259 vagina. NEW 260 2.1.2 Vesico-vaginal fistula (VVaF): abnormal connection between the bladder and the 261 vagina. NEW 262 2.1.3 Vesico-uterine fistula (VUF): abnormal connection between the bladder and the 263 uterus. NEW 264 2.1.4 Uretero-vaginal fistula (UrVaF): abnormal communication between the ureter and 265 the vagina. NEW 266 2.1.5 (Colo)-Recto-vaginal fistula (RVaF): abnormal connection between the rectum 267 (colon) and the vagina. NEW 268 2.1.6 (Colo)-Rectal to Urinary Tract: abnormal connection between the rectum (colon) 269 and any part of the urinary tract. NEW

270 271 Figure 1: Basic pelvic floor fistula anatomy. © Levent Efe 272 273 2.2 URETHRO-VAGINAL FISTULA (UVF)

274 2.2.1 Partial urethro-vaginal fistula (UVaF): urethral structure is evident, with a 275 demonstrable fistula defect (Fig 2). NEW 276

277 278 Figure 2: Urethro-vaginal fistula demonstrated by metal catheter – 1cm above the 279 external urethral meatus. © J Goh (above) © Levent Efe (below) 280 281 2.2.2 Total urethro-vaginal fistula (UVaF): urethral structure is not evident (Fig 3). 282 NEW 283 2.2.3 Circumferential fistula (genito-urinary): an entire segment (anterior, 284 posterior, lateral urethra) from anterior vaginal wall to the posterior aspect of the 285 pubic symphysis is absent and destroyed23,24 . The circumferential fistula almost always 286 involves the urethra and the fistula totally separates the proximal urethra/bladder 287 from the distal portion (Fig 4). Bladder involvement with a circumferential fistula is 288 common. NEW

Absent urethra

289 290 291 Figure 3A (left): Total urethro-vaginal fistula (UVaF) – total absence of anterior vaginal wall 292 and posterior urethra from external urinary meatus to bladder neck. © J Goh. Fig 3B: (right) 293 © Levent Efe

Urethral/distal part of fistula

Circumferential defect

294 295 296 297 Figure 4A (left): Circumferential fistula – an entire segment of urethra (anterior, lateral, 298 posterior) and anterior vaginal wall is absent. Proximal (bladder) part of the fistula is 299 completely disconnected from the distal (urethra) portion. © J Goh Fig 4B: (right) © Levent 300 Efe

301 302 Fig 5A (left): Vesico-vaginal fistula (VVaF): metal catheter inserted in urethra visible through 303 vesico-vaginal fistula (VVaF). © J Goh; Fig 5B: (right) © Levent Efe 304 305 2.3 VESICO-VAGINAL FISTULA (VVF): 306 2.3.1 Vesico-vaginal fistula (VVaF): fistula affecting anterior vaginal wall and 307 posterior bladder wall with or without involvement of the ureteric orifices (Fig 5A,5B). 308 NEW 309 2.3.2 Circumferential fistula (genito-urinary): see above 2.2.3. It almost always 310 involves the urethra. NEW 311 2.3.3 Vesico-vaginal vault fistula (VVtF): vesico-vaginal fistula located at vaginal 312 vault (cuff) following hysterectomy (Fig 6A,B). NEW

313 314 315 Figure 6A (left): Vesicovaginal vault fistula (VVtF) after hysterectomy © J Goh; Fig 6B (right): 316 © Levent Efe 317 318 319 2.4 VESICO-UTERINE FISTULA (VUF) 320 2.4.1 Vesico-cervical fistula: abnormal connection between the bladder and the 321 cervix. May occur after caesarean section, procedures to the cervix, supra-cervical 322 hysterectomy. NEW 323 2.4.2 Vesico-uterine fistula: abnormal connection between the bladder and the 324 body of the uterus. NEW 325 326 2.5 URETERO-VAGINAL FISTULA (UrVaF) 327 2.5.1 Uretero-vaginal fistula (UrVaF): abnormal connection between the ureter and 328 the vagina. NEW 329 2.5.1.1 Uretero-vaginal fistula (UrVaF) may be congenital (ectopic ureter) NEW 330 or 331 2.5.1.2 Acquired (e.g. following surgery or obstructed labor) NEW

332 2.5.2 Uretero-vesical-vaginal fistula (UrVVaF): fistula involving the ureter(s), 333 bladder and vagina. This may be seen with a large 334 obstetric fistula and the ureter is outside the VVF. NEW 335 2.5.3 Uretero-uterine (cervical) fistula (UrUF/ UrCxF): 336 abnormal connection between the ureter and the 337 uterus (cervix). Predominantly post-caesarean or post- 338 hysterectomy. NEW 339 340 2.6 PELVIC FLOOR FISTULA – ANORECTAL TRACT TO 341 VAGINA (UTERUS) 342 2.6.1 Fourth-degree tears: obstetric anal sphincter injury with disruption of the 343 perineal body, connecting the vagina to the anorectum. The internal and external anal 344 sphincters are disrupted. NEW 345 2.6.1.1 Acute fourth degree tear – occurs at time of childbirth or other 346 trauma. NEW 347 2.6.1.2 Chronic fourth degree tear – unrepaired or dehiscence 348 following repair at time of childbirth or other trauma, resulting in an absent

349 perineal body with a total perineal defect FN2.1 (Fig 7 A, B). NEW 350 351 352 353 354 355 356 357

358 359 Fig 7A: Fourth degree rectovaginal tear with perineal body disruption. Congenital defects of 360 a similar configuration may also occur. © J Goh; Fig 7B: © Levent Efe 361 2.6.2 Recto-vaginal fistula (RVaF): abnormal connection between the rectum and 362 the vagina. NEW 363 2.6.2.1 Non-circumferential recto-vaginal fistula (RVaF): involves the 364 posterior vaginal wall and anterior rectum. NEW

365 2.6.2.2 Circumferential recto-vaginal fistula (RVaF): involves an entire

366 segment of the rectum, involving the posterior vaginal wall, anterior and

367 posterior rectum. The proximal rectal part of the fistula is completely

368 separated from the distal portion. NEW

369 2.6.2.3 Rectal/vaginal/perineal fistula (RVaPeF) : Is an abnormal 370 communication from the anorectum to the vagina or perineal area. NEW

371 2.6.3 Recto-uterine- cervical fistula (RUF/RCxF) – abnormal connection from the 372 rectum to the uterus or cervix. NEW 373 2.6.4 Fistula in ano (FIA) / Ano-cutaneous fistula (ACF) An anal fistula is an 374 abnormal connection between the anal canal epithelium and the skin epithelium. 375

376 2.7 PELVIC FLOOR FISTULA – (COLO) RECTAL TO URINARY TRACT 377 2.7.1 Colo-vesical fistula (CoVF): abnormal connection between the rectum (colon) 378 and the bladder. NEW 379 2.7.2 Recto (colo)- ureteric fistula (CoUrF / RUrF): abnormal connection between

380 the rectum (colon) and the ureter FN 2.2. NEW 381 382 2.8 PUBLISHED CLASSIFICATION SYSTEMS OF PELVIC FLOOR FISTULA 383 There are published classification systems used for female pelvic floor fistulas predicated on 384 and devised from their ability to predict outcomes of surgery based on these classification

385 systems FN2.3 - FN2.7. These classification systems are: (i) the Francophone System; (ii) the 386 Waaldjik System; (iii) the Goh System; (iv) the Panzi Hospital System.

387 Footnotes Section

388 FN 2.1. Total perineal defect12: A spectrum of tissue loss from the perineal body and 389 rectovaginal septum with variable appearance. 390 FN 2.2. Recto(colo)-ureteric fistula is created electively after ureteric diversion into the bowel 391 for the management of PFF but can occur following colorectal surgery for cancer and 392 inflammatory pathologies. 393 FN 2.3. There are multiple classification systems published. Section 2.8 briefly mentions the 394 more commonly used systems. Commonly used anatomical descriptions of PFF such as 395 ‘urethral’, ‘mid-vaginal’ and ‘juxta-cervical’ are terms from various published classification 396 systems (see Goh et al23,24 for a more extensive review).There is currently no consensus on a 397 classification for PFF and a comprehensive review on published classification systems was 398 undertake previously23. Below are commonly used PFF classifications. 399 FN 2.4 Classification System A: The Francophone System 24, 25, developed in 1959, has been 400 for use in urinary tract PFF and is used in Francophone Africa. It divides the fistula into 401 ‘simple’, ‘complex’ or complicated with significance placed on destruction of bladder neck, 402 urethra and scarring. It is the original classification system that was translated into English to 403 create the basis for the Waaldijk classification system.

404 FN 2.5 Classification System B: The Waaldijk System26: published in 1995, it is based on 405 whether the continence mechanism is impaired and on the extent of circumferential damage.

406 In the originating paper, the classification of the fistula was performed under anesthesia. Type 407 I fistulas do not involve ‘the closing mechanism’ whilst Type II involves “the closing 408 mechanism”. The definition of the ‘closing mechanism’ is unclear. Type III are ureteric and 409 ‘other exceptional fistulas’. There is a subclassification according to the size of the fistula. 410 Studies have been conducted to assess this system. Comparative study with other systems 411 demonstrates the Waaldijk system to be less predictive of closure.

412 FN 2.6 Classification System C: The Goh System27: published in 2004, this system is based on 413 fixed reference points. The external urinary meatus (or its site if the urethra is absent) is the 414 reference point for genito-urinary fistulas and the hymen is the reference point for anorectal- 415 vaginal fistulas. This system is based on distance from these fixed reference points, size of the 416 fistula, presence of scarring and other ‘special’ circumstances such as radiation fistulas, 417 circumferential fistulas, recurrent fistulas. Published studies using this system include intra- 418 and inter-observer concordances28, correlations with urinary incontinence after surgical 419 closure and grade of fistula29 and comparative studies with other systems30. 420 421 FN 2.7 Classification System D: The Panzi Hospital System31: published in 2018: also known 422 as the Panzi score, is a descriptive and predictive scoring system based on retrospective 423 review of surgical failure of fistula repair using characteristics from the Goh27 and Waaldjik26 424 systems. A scoring system was constructed by using the data obtained, correlating the Score 425 to likelihood of surgical outcomes. The Score is based on whether the fistula is 426 circumferential, the location and size of the fistula. 427

428

429 SECTION 3: SYMPTOMS

430 Symptom: Any morbid phenomenon or departure from the normal in structure, function or 431 sensation, experienced by the woman and indicative of disease or a health problem1,2. 432 Symptoms are either volunteered by or elicited from the woman or may be described by the 433 woman’s caregiver. 434 435 Fistula symptoms: A departure from normal sensation, structure or function, reported by a 436 woman as: (i) leakage of urine and/or faeces or flatus from the vagina or perineum or; (ii) less

437 commonly as leakage of urine from the anus, or cyclic menouria or haematuria from the 438 urinary tract; or (iii) menstrual flow or other cyclic blood per anum/rectum. Symptoms are 439 often, but not always, continuous, severe and may vary with position including leakage when 440 sleeping (supine). Fistulas with a long tract or flap valve or small defect may make symptoms 441 intermittent NEW. 442 443 3.1 GENITAL TRACT FISTULA SYMPTOMS 444 3.1.1 Discomfort or pain: complaint of discomfort/pain on the vulva, buttocks, thigh 445 or legs due to urine or faecal irritation, with or without ulceration or bleeding. NEW 446 3.1.2 Vaginal urine leakage: complaint of urine leakage through the vagina. 447 Symptoms are usually continuous but may be intermittent and may be associated with 448 movement or specific changes of position. NEW 449 3.1.3 Vaginal flatus/faeces: complaint of passage of flatus or faeces per vaginam12. 450 Symptoms are usually continuous but may be intermittent and may be associated with 451 movement or specific changes of position NEW 452 3.1.4 Hematuria: Complaint of the passage of visible blood mixed with urine2. 453 454 3.2 URINARY TRACT FISTULA SYMPTOMS 455 3.2.1 Urinary incontinence: complaint of involuntary loss of urine1-3,6-7. 456 3.2.2 Continuous (urinary) incontinence: complaint of continuous involuntary loss 457 of urine1-3,6-7. 458 3.2.3 Postural (urinary) incontinence: Complaint of involuntary loss of urine 459 associated with change of body position, for example, rising from a seated or lying 460 position1,3. 461 3.2.4 Nocturnal enuresis: Complaint of involuntary loss of urine which occurs during 462 the main sleep period32. 463 3.2.5 Insensible (urinary) incontinence: Complaint of urinary incontinence where 464 the woman is aware of urine leakage but unaware of how or when it occurred.33 465 3.2.6 Coital incontinence: Complaint of involuntary loss of urine during or after 466 vaginal intercourse14. This symptom might be further divided into that occurring with 467 penetration or intromission and that occurring at orgasm.

468 3.2.7 Menouria: Complaint of cyclic haematuria that the patient believes to be 469 menstrual. It may represent a vesico-uterine fistula. NEW 470 471 3.3 ANORECTAL TRACT FISTULA SYMPTOMS

472 3.3.1 Anal incontinence (symptom): complaint of involuntary loss of flatus or feces 473 1, 12 474 3.3.2 Fecal incontinence: Complaint of involuntary loss of feces. 475 3.3.2.1 Solid1,12 476 3.3.2.2 Liquid1,12 477 3.3.3 Flatal Incontinence: Complaint of involuntary loss of flatus (gas) 12 478 3.3.4 Double incontinence: Complaint of both anal incontinence and urinary

479 incontinence12. 480 3.3.5 Coital fecal (flatal) incontinence: Fecal (flatal) incontinence occurring with 481 vaginal intercourse12 482 3.3.6 Passive fecal leakage: Involuntary soiling of liquid or solid stool without 483 sensation or warning or difficulty wiping clean12. 484 3.3.7 Overflow faecal incontinence: Seepage of stool due to an overfull rectum or 485 fecal impaction12. 486 3.3.8 Nocturnal defecation: Complaint of interruption of sleep one or more times 487 because of the need to defecate12. 488 3.3.9 Flaturia: Complaint of passage of gas per urethra12. 489 3.3.10 Fecaluria: Complaint of passage of fecal material (per urethra) in the urine2. 490 3.3.11 Rectal leakage of menses: Complaint of blood or bloody discharge passing per 491 anus that the patient believes to be menstrual. NEW 492 3.3.12 Rectal leakage of urine: Complaint of urine passing per anus. NEW 493 494 3.4 CHRONIC FISTULA SYMPTOMS 495 3.4.1 Persistent fistula (symptom): Continuation of urinary tract and/or anorectal 496 tract incontinence symptoms immediately after fistula treatment caused by 497 incomplete fistula wound healing. This includes inability to close the fistula during 498 surgery. NEW

499 3.4.2 Recurrent fistula (symptom): Recurrence of fistula defect and incontinence 500 after a period of transient complete fistula wound healing followed by delayed 501 complications of wound healing causing fistula breakdown and fistula re-formation. 502 NEW 503 It may also be caused by a new index event within the interval from successful repair 504 to recurrence of fistula after which another fistula forms. Examples of subsequent 505 index events include subsequent pregnancy complications causing obstetric PFF, 506 pelvic floor surgery complicated by iatrogenic PFF, malignancy or pelvic trauma 507 causing traumatic PFF 508 3.4.3 Post-repaired fistula residual incontinence (RI) symptoms: Urinary or

509 anorectal tract incontinence symptoms after successful fistula closure NEW FN 3.1 510 511 3.5 PERSISTENT FISTULA-RELATED DISORDER (PFRD) SYMPTOMS: symptoms from 512 conditions concurrent with the fistula or occurring after successful closure of the fistula 513 defect. PFRD may include a complex of disabling symptoms related to co-morbidities of 514 general health and well-being, mental, reproductive, and musculoskeletal organs, in addition 515 to symptoms from disorders of the upper and lower urinary, genital and anorectal tracts. NEW 516 Co-morbidities include but not limited to: 517 3.5.1 PFRD pain: e.g. pain or discomfort in the vagina or vulva with sexual activity. NEW W 518 3.5.2 PFRD mobility dysfunction symptoms: Difficulty walking or changing position or other 519 range of motion symptoms. NEW 520 3.5.3 PFRD menstrual dysfunction symptoms: Amenorrhea, oligomenorrhea, dysmenorrhea, 521 infertility. NEW 522 3.5.4 PFRD urinary tract dysfunction symptoms: e.g. Flank pain, dysuria, haematuria, 523 voiding dysfunction NEW 524 3.5.5 PFRD psychological dysfunction symptoms: Anxiety, depression, adjustment 525 disorder with depressed mood, mourning or grieving may be due to the impact of body 526 image. Effects of loss of income-generating potential or marital, family or social status. NEW 527 N.B. This terminology document will restrict detailed PFRD terminology definitions to urinary, 528 genital and anorectal tract for the remainder of the document. 529 530 3.5.6 Other common PFRD symptoms

531 3.5.6.1 General health symptoms35: NEW 532 3.5.6.1.1 Fatigue, malaise, and mental health symptoms which are often multi- 533 factorial in origin 534 3.5.6.1.2 Emotional, musculoskeletal, gastrointestinal, or urinary tract 535 symptoms related to types of abuse – physical, economic and/or emotional 536 3.5.6.2 Mental health symptoms36-38 : NEW 537 3.5.6.2.1 Anxiety and/or depression, post-traumatic stress disorder 538 3.5.6.2.2 Grieving/mourning, stigma and social isolation, self-esteem, 539 quality of life 540 3.5.6.2.3 Suicidal ideation, loss of libido, body image disorders, dysphoria, 541 insomnia 542 3.5.6.3 Musculo-skeletal symptoms35,36,39: NEW 543 3.5.6.3.1 Difficulty with ambulation 544 3.5.6.3.2 Complaint of other quality of life challenges related to activities 545 of daily living caused by diastasis pubis, osteomyelitis, foot-drop, levator ani 546 atrophy, exposed sacral nerve roots, idiopathic chronic pelvic pain or other 547 musculoskeletal condition incident after index event causing the fistula. 548 3.5.6.4 Reproductive health symptoms36: NEW 549 3.5.6.4.1 Amenorrhea, oligomenorrhea, dysmenorrhea 550 3.5.6.4.2 Infertility 551 552 3.5.7 Women deemed incurable (WDI) Women with primary, persistent and recurrent fistula 553 for which anatomic repair is not possible. WDI require either supportive management and/or 554 a diversion procedure, or they have a fistula complexity that exceeds the capacity(s) of the

555 highest available surgical facility: FN 3.2 NEW 556

557 3.6 PFRD symptoms of the urinary tract may include:

558 3.6.1 PFRD Sensory urinary tract symptoms: A departure from normal sensation or 559 function, experienced by the woman during bladder filling. Normally, the individual is aware 560 of increasing sensation with bladder filling up to a strong desire to void1.

561 3.6.1.1 Increased urinary frequency: Complaint that voiding occurs more

562 frequently than deemed normal by the individual (or caregivers). Time of day 563 (daytime or nocturnal or number of voids are not specified) 564 3.6.1.2 Increased bladder sensation: Complaint that the desire to void during 565 bladder filling occurs earlier or is more persistent to that previous experienced. 566 This differs from urgency by the fact that micturition can be postponed 567 despite the desire to void1. 568 3.6.1.3 Reduced bladder sensation: Complaint that the definite desire to 569 void occurs later to that previously experienced despite an awareness that the 570 bladder is filling1. 571 3.6.1.4 Absent bladder sensation: Complaint of both the absence of the 572 sensation of bladder filling and of a definite desire to void1. 573 3.6.2 PFRD Voiding and Postmicturition Symptoms: A departure from normal 574 sensation or function, experienced by the woman during or following the act of 575 micturition1 576 3.6.2.1 Hesitancy: Complaint of a delay in initiating micturition1. 577 3.6.2.2 Slow stream: Complaint of a urinary stream perceived as slower 578 compared to previous performance or in comparison with others1. 579 3.6.2.3 Intermittency: Complaint of urine flow that stops and starts on one or 580 more occasions during voiding1. 581 3.6.2.4 Straining to void: Complaint of the need to make an intensive effort 582 (by abdominal straining, Valsalva or suprapubic pressure) to either initiate, 583 maintain, or improve the urinary stream1. 584 3.6.2.5 Spraying (splitting) of urinary stream: Complaint that the urine passage 585 is a spray or a split stream rather than a single discrete stream1. CHANGED 586 3.6.2.6 Feeling of incomplete (bladder) emptying: Complaint that the bladder 587 does not feel empty after micturition. 588 3.6.2.7 Need to immediately re-void: Complaint that further micturition is 589 necessary soon after passing urine1. 590 3.6.2.8 Postmicturition leakage: Complaint of a further involuntary passage or 591 loss of urine following the completion of micturition1. CHANGED 592 3.6.2.9 Position-dependent micturition: Complaint of having to take specific 593 positions to be able to micturate spontaneously or to improve bladder emptying,

594 for example, leaning forwards or backwards on the toilet seat or voiding in the semi- 595 standing position1,3. 596 3.6.2.10 Dysuria: Complaint of burning or other discomfort during micturition. 597 Discomfort may be intrinsic to the lower urinary tract or external (vulvar dysuria)1. 598 3.6.2.11 Urinary retention: Complaint of the inability to pass urine despite 599 persistent effort1. 600 3.6.3 PFRD Lower Urinary Tract Infection Symptoms:

601 3.6.3.1 Urinary tract infection (UTI): Defined as microbiological evidence of 602 significant bacteriuria and pyuria usually accompanied by symptoms such as 603 increased bladder sensation, urgency, frequency, dysuria, urgency urinary 604 incontinence, and/or pain in the lower urinary tract. 605 3.6.3.2 Recurrent urinary tract infections (UTIs): At least three symptomatic 606 and medically diagnosed UTI in the previous 12 months. The previous UTI(s) should 607 have resolved prior to a further UTI being diagnosed. 608 3.6.3.2.1 Other related history: hematuria, catheterization. 609 3.6.4 PFRD Lower Urinary Tract Pain Symptoms:

610 3.6.4.1 Bladder pain: Complaint of suprapubic or retropubic pain, pressure, 611 or discomfort, related to the bladder, and usually increasing with bladder filling. It 612 may persist or be relieved after voiding1,3. 613 3.6.4.2 Urethral pain: Complaint of pain felt in the urethra and the woman 614 indicates the urethra as the site1,3. 615 616 3.7 PFRD Pelvic Organ Prolapse (POP) symptoms3: A departure from normal 617 sensation, structure, or function, experienced by the woman in reference to the position of 618 her pelvic organs. Symptoms are generally worse at the times when gravity might make 619 the prolapse worse (e.g., after long periods of standing or exercise) and better when 620 gravity is not a factor (e.g. lying supine). Prolapse may be more prominent at times of 621 abdominal straining, for example, defecation. Other associated terms include: 622 3.7.1 Vaginal bulging: Complaint of a ‘‘bulge’’ or ‘‘something coming down’’ 623 towards or through the vaginal introitus3. 624 3.7.2 Vaginal gaping: Complaint of a “wide open” vaginal introitus. NEW

625 3.7.3 Pelvic pressure: Complaint of increased heaviness or dragging in the 626 suprapubic area and/or pelvis1,3. 627 3.7.4 Bleeding, discharge, infection: Complaint of vaginal bleeding, discharge, or 628 infection related to prolapse3. 629 3.7.5 Splinting/digitation: Complaint of the need to digitally replace the 630 prolapse3 or to avoid prolapse descent during periods of increased abdominal 631 pressure. CHANGED 632 3.7.6 Low backache: Complaint of low, sacral (or ‘‘period-like’’) backache 633 associated with POP3 634 635 3.8 PFRD Sexual Dysfunction Symptoms1,14: A departure from normal 636 sensation and/or function experienced by a woman during sexual activity. 637 3.8.1 Dyspareunia: Complaint of persistent or recurrent pain or discomfort 638 associated with attempted or complete vaginal penetration.1,14 639 3.8.2 Superficial (introital) dyspareunia: Complaint of pain or discomfort on 640 vaginal entry or at the vaginal introitus.1,14 641 3.8.3 Deep dyspareunia: Complaint of pain or discomfort on deeper penetration 642 (mid or upper vagina).1, 643 3.8.4 Obstructed intercourse: Complaint that vaginal penetration is not possible due 644 to obstruction.14 645 3.8.5 Vaginal laxity: Complaint of excessive vaginal laxity14. 646 647 3.9 PFRD Genital Pain Symptoms1, 14: 648 3.9.1 Vulval pain: Complaint of pain felt in and around the vulva14. 649 3.9.2 Vaginal pain: Complaint of pain felt internally within the vagina, above the 650 Introitus1,14. 651 3.9.3 Perineal pain: Complaint of pain felt between the posterior fourchette 652 (posterior lip of the introitus) and the anus14. 653 3.9.4 Pelvic pain: The complaint of pain perceived to arise in the pelvis14. 654 3.9.5 Cyclical (menstrual) pelvic pain: Cyclical pelvic pain related to menses that 655 raises the possibility of a gynaecological cause14. 656 3.9.6 Pudendal neuralgia: Burning vaginal or vulval (anywhere between anus and 24

657 clitoris) pain associated with tenderness over the course of the pudendal nerves14. 658 3.9.7 Chronic lower urinary tract and/or other pelvic pain syndromes1: 659 660 3.10 PFRD anorectal tract symptoms1,12: 661 3.10.1 Straining to defecate: Complaint of the need to make an intensive effort (by 662 abdominal straining or Valsalva) to either initiate, maintain, or improve defecation1,12. 663 3.10.2 Feeling of incomplete (bowel) evacuation: Complaint that the rectum does 664 not feel empty after defecation12. 665 3.10.3 Diminished rectal sensation: Complaint of diminished or absent sensation in 666 the rectum12. 667 3.10.4 Constipation: Complaint that bowel movements are infrequent and/or 668 incomplete and/or there is a need for frequent straining or manual assistance to 669 defecate.12 670 3.10.5 Rectal prolapse: Complaint of external protrusion of the rectum12. 671 3.10.6 Rectal bleeding/mucus: Complaint of the loss of blood or mucus per rectum12. 672 3.10.7 Pain during straining/defecation: Complaint of pain during defecation or 673 straining to defecate.12 674 3.10.8. Levator ani syndrome: Episodic rectal pain caused by spasm of the levator ani 675 muscle. Proctalgia fugax (fleeting pain in the rectum) and coccydynia (pain in the 676 coccygeal region) are variants of levator ani syndrome.12 677 3.10.9 Proctalgia fugax is a severe, episodic, generally sacrococcygeal pain.12 678 3.10.10 Fecal incontinence: Involuntary loss of feces or flatus1,2 679 680 681 682 Footnotes Section 3 683 FN 3.1 About 1 in 4 women complains of ongoing urinary incontinence after successful fistula 684 closure29. Urodynamic studies were performed in 149 women with post-fistula 685 incontinence34. The most common diagnoses were urodynamic stress incontinence in 49%, 686 mixed urodynamic stress incontinence and detrusor overactivity in 43%. Seven percent of 687 women had a post-void residual urine of 150 mL or more (which is high and significant, 688 particularly in a partially destroyed bladder that has a maximum capacity of 150ml)

689 690 FN 3.2 Women deemed incurable: In some facilities, this includes women with severe 691 incontinence symptoms after successful fistula closure. These women also fall under “Closed 692 and Incontinent” category. 693

694 SECTION 4: PELVIC FLOOR FISTULA (PFF) SIGNS

695 4.1 GENERAL PRINCIPLES OF PFF SIGNS

696 4.1.1 Sign: Any abnormality indicative of disease or a health problem, discoverable on 697 examination of the patient; an objective indication of disease or a health problem1.

698 4.1.2 Correlation of signs and symptoms: signs should correlate with symptoms e.g. patient 699 report of urinary incontinence is corroborated by visualization of urine leakage into the 700 genital tract through a fistula defect.

701 4.1.3 Overlap of PFF and non-PFF signs: Because the signs of pelvic floor fistulas overlap with 702 symptoms of urinary and faecal incontinence in patients who have never had a fistula, 703 detailed pelvic exam is essential. Fill tests, with or without dye, may also be used during 704 physical examination to assess the defect(s). The aim is to firstly diagnose the fistula(s) and to 705 identify the location of the fistula(s) and then to assess the injury by evaluating the amount

706 of tissue defect and scarring/fibrosis. FN 4.1

707 4.1.4 General examination: is fundamental to the surgical triage process in order to assure 708 that patients undergoing fistula surgery are suitable for anesthetic and surgical intervention. 709 Surgery scheduling should be delayed until underlying conditions are stabilized with 710 treatment to the best possible state of health. General examination must also rigorously 711 screen for any condition that will impair optimal wound healing, so that the condition may be 712 treated, or cured, before elective reconstructive fistula surgery. Signs of conditions relevant 713 for elective reconstructive surgical triage screening include amongst others: anaemia, 714 malnutrition, diabetes, malaria, and other parasites, hepatitis, hypertension, rehydration, 715 renal dysfunction, STI and HIV.

716

717 4.2 VAGINAL FISTULA SIGNS

718 4.2.1 Vaginal leakage: urine, flatus and/or stool observed leaking into the vagina or from the 719 vagina. NEW

720 4.2.2 Excoriation: skin excoriation and/or rash with or without crusting or scabbing on the 721 tops (or soles as urine pools in plastic sandals) of feet, inner thighs, external genitalia (Fig 8), 722 perineum or vagina12. CHANGED

723

724 Fig 8: Vulvar dermatitis from exposure to urine © J Goh

725 4.2.3 Bleeding, discharge: observed on vaginal examination of the fistula. This includes 726 hematoma. NEW

727 4.2.4 Scars, sinuses, deformities: vaginal scarring, vaginal sinus tracts, vaginal stenosis. NEW

728

729 4.3 URINARY TRACT PFF SIGNS

730 4.3.1 Extra urethral incontinence: Observation of urine leakage through channels other 731 than the urethral meatus, e.g. fistula12. The fistula may be described anatomically from one 732 structure to another. Below are anatomical descriptions of PFF. The PFF defects may occur 733 between 2 or more structures.

734 4.3.2 LOWER URINARY TRACT PFF FN 4.2

735 4.3.2.1 Urethro-vaginal fistula (UVaF) – clinical exam only: observation of a defect 736 between the urethra and vagina that may occur across a spectrum of tissue loss, from 737 the urethral meatus to the level of the bladder neck, with variable appearance. (Fig 2

738 & 3) FN4.3 NEW With or without observation of:

739 4.3.2.1.1 UVaF – Clinical exam and probe: Probe passing though urethra into the 740 vagina through a urethral defect or from the urethral defect back out through the 741 urethral meatus. NEW

742 4.3.2.1.2 UVaF – Clinical exam and fluid instillation: dyed irrigant fluid passing per 743 defect at the time of retrograde fill test of the bladder through a bladder catheter 744 (positive blue test) (Fig 9). NEW

745 4.3.2.1.3 UVaF – Clinical exam and Trattner catheter: Trattner catheter (Fig 9) may 746 be used to isolate retrograde blue test filling to the urethral lumen without filling the 747 bladder. NEW

748

749 Figure 9: Trattner double balloon urethral catheter demonstrating retrograde blue 750 dye for detection of small urethral fistula.

751 4.3.2.2 Vesico-vaginal fistula (VVaF) – clinical exam only: observation of urine pooling 752 in the vagina and observation of defect between the anterior vaginal wall (including 753 vault) and the bladder (Fig 5). NEW With or without observation of:

754

755

756 Fig 10A: Retrograde blue test positive for vesico-vaginal fistula (VVaF) © L J Romanzi; Fig 757 10B: © Levent Efe 758 4.3.2.2.1 VVaF – Clinical exam plus probe: Probe passing though urethra into

759 the vagina or from the vagina through the urethral meatus (Fig 11). NEW

760 4.3.2.2.2 VVaF – Clinical exam plus irrigation: Dyed irrigation fluid passing

761 per defect at the time of retrograde fill test of the bladder through a bladder

762 catheter (positive blue test). NEW

763 4.3.2.2.3 VVaF – Clinical exam plus bladder mucosa seen: Bladder mucosa

764 visible through the vagina on speculum examination (Fig 6) NEW

765

766 4.3.2.3 Vesico-uterine(cervical) fistula (VUF /VCxF)): defect between the uterus 767 (and/or cervix) and bladder, where the cervix may be intact or deficient. NEW with or 768 without observation of:

769 4.3.2.3.1 VUF – Clinical Exam only: Menouria: (cyclical) haematuria coinciding

770 with menstruation. NEW

771 4.3.2.3.2 VUF – Clinical exam plus probe: Probe passing though urethra into

772 the cervical os or from the cervix through the urethral meatus (Fig 11). NEW

773 4.3.2.3.3 VUF – Clinical exam plus irrigation: Dyed irrigation fluid passing

774 per cervical os at the time of retrograde dyed irrigant fill test of the

775 bladder through a bladder catheter. NEW

776

777

778

779 Figure 11A (above): Vesico-cervical fistula (: Metal catheter inserted per urethra is 780 visible within the cervical os. © J Goh; 11B (below) © Levent Efe

781 4.3.2.4 Colo-vesical) fistula (CoVF): Defect between the anorectum (or colon) and 782 bladder. NEW with or without observation of:

783 4.3.2.4.1 CoVF -Clinical exam only: observation of flaturia, faecaluria. NEW

784 4.3.2.4.2 CoVF - Clinical exam plus PR air injection: observation of flaturia,

785 fecaluria bubbles passing through the urethra after retrograde injection of air

786 per rectum. NEW

787 4.3.2.4.3 CoVF Clinical exam plus irrigation: observation of dyed

788 irrigation fluid passing per anorectum after retrograde bladder fill per urethra.

789 NEW

790 4.3.3 UPPER URINARY TRACT PFF

791 4.3.3.1 Uretero-vaginal fistula (UrVaF): defect between the ureter(s) and vagina.

792 NEW With or without observation of:

793 4.3.3.1.1 UrVaF - Clinical exam only: observation of urine pooling in the 794 posterior vaginal fornix. NEW

795 4.3.3.1.2 UrVaF – Clinical exam plus irrigation: observation of urine

796 pooling in the posterior vaginal fornix at the time of retrograde dyed

797 irrigation fill test of the bladder through a bladder catheter (negative dye

798 test, positive urine). NEW

799 4.3.3.1.3 UrVaF – occurrence in isolation: In isolation e.g. at the vaginal vault 800 following a hysterectomy including Caesarean hysterectomy. NEW

801 4.3.3.1.4 UrVaF – occurrence in combination: e.g. in combination of a

802 vesico-vaginal fistula (VVaF) NEW

803

804 4.3.3.2 Uretero-uterine(cervical) fistula (UrUF / UrCxF): defect between the ureter(s) 805 and the cervix. NEW With or without observation of:

806 4.3.3.2.1 UrUF - Clinical exam only: observation of urine passing

807 through the cervix or pooling in the posterior vaginal fornix. NEW

808 4.3.3.2.2 UrUF – Clinical exam plus irrigation: observation of urine

809 passing per cervical os; with or without pooling in the posterior vaginal fornix

810 at the time of retrograde dyed irrigant fill test of the bladder through a

811 bladder catheter (negative blue test, positive clear urine). NEW

812 4.3.3.3 Uretero-uterine(cervico)-vesical fistula: Complex of multiple urinary tract 813 fistulas concurrent between the ureter and uterus/cervix and between the 814 bladder and uterus/cervix. NEW Difficult to diagnose clinically. It is often

815 diagnosed by hystero-salpingogram (HSG) FN4.6.

816 4.4 ANORECTO-VAGINAL FISTULA SIGNS12

817 4.4.1 General signs

818 4.4.1.1 Excoriation dermatitis: inner thighs, external genitalia, generally

819 4.4.1.1.1 Perineum or vagina with or without skin rashes, crusting or scabbing. 820 CHANGED

821 4.4.1.2 Soiling: perianal, vaginal or perineal faecal soiling1,12

822 4.4.1.3 Discharge: perianal or vaginal bloody or mucus discharge1,12

823 4.4.1.4 Scars, sinuses, deformities, hematoma1,12

824

825 4.4.2 Deficient perineum/ total perineal defect: A spectrum of tissue loss from the perineal 826 body and rectovaginal septum with variable appearance. There can be a common cavity made 827 up of the anterior vagina and posterior rectal walls or just an extremely thin septum between 828 the anorectum and vagina12

829 4.4.3 Fourth degree perineal tear (40PT): defined as an acquired childbirth injury and a subset 830 of deficient perineum, involving both loss of the rectovaginal septum, full thickness anterior 831 defect of the anal sphincter, and variable loss with lateral displacement of the fibromuscular 832 architecture of the perineal body (total perineal defect) (Fig 7)

833 4.4.4 Rectovaginal fistula (RVaF): Defect between the rectum to the vagina with or without 834 observation of vaginal flatus/feces12. With or without the observation of:

835 4.4.4.1 RVaF – Clinical exam only: Anorectal fluid per vagina. NEW

836 4.4.4.2 RVaF = Clinical exam plus probe: Probe or examination finger passing per 837 vagina through anus or per anus through vagina (Fig 12). NEW

838

839 Figure 12A: Recto-vaginal fistula (RVaF), low in the vagina, just proximal to the anus ©J Goh; 840 12B: © Levent Efe 841 842 4.4.4.3 RVF – Clinical exam plus irrigation or air injection: Anorectal tract fluid per

843 vaginam, or with bubbles passing through the defect through vaginal irrigant fluid

844 after retrograde injection of air per rectum NEW

845 4.4.5 Colo-uterine/cervical fistula (CoUF /CoCxF): Defect between the colo/rectum and 846 uterus (body and/or cervix). NEW with or without the observation of:

847 4.4.5.1 R(C)UF – Clinical exam only: passing flatus/faeces per cervix, menses per

848 rectum, anorectal tract fluid per vagina. NEW

849 4.4.5.2 R(C)UF – Clinical exam plus irrigation or air injection: with bubbles passing through 850 the defect through vaginal irrigant fluid after retrograde injection of air per rectum. NEW

851 4.4.6 Rectal/vaginal/perineal fistula (RVaPeF) : Is an abnormal communication from the 852 anorectum to the vagina or perineal area. NEW

853 4.4.6.1 RVaPeF – Clinical Exam only: Passing of flatus/feces per vagina or perineum

854 4.4.6.2 RVaPeF – Clinical exam plus probe: Probe passing per vagina or perineum

855 through anus

856 4.4.7. Vesico-rectal fistula (VRF): Defect between the bladder and rectum. NEW with or 857 without observation of:

858 4.4.7.1 VRF – clinical exam plus probe: probe passing per urethra through anus or per 859 anus through urethra. NEW

860 4.4.7.2 VRF – clinical exam plus irrigation: Flaturia, fecaluria, bubbles passing through 861 the urethra after retrograde injection of air per rectum, blue irrigant fluid passing per 862 anorectum after retrograde bladder fill per urethra. NEW

863 4.4.8 Fistula in ano (FIA) / Ano-cutaneous fistula (ACF)An anal fistula is an abnormal 864 connection between the anal canal epithelium and the skin epithelium.

865 4.4.8.1 Patients may complain of pain, swelling, intermittent discharge of blood or pus from 866 the fistula, and recurrent abscesses formation12 .

867 4.5 CHRONIC FISTULA SIGNS

868 4.5.1 Persistent fistula : The persistent fistula is not de novo to the patient

869 4.5.1.1: Persistent urine or fecal (flatal) incontinence: Observation of 870 involuntary, extra-urethral loss of urine and/or extra-anal loss of flatus/feces on 871 examination . NEW

872 4.5.1.2 Incomplete fistula wound healing: after treatment which includes 873 inability to close the fistula during surgery. NEW

874 4.5.2 Recurrent fistula (signs): The recurrent fistula is de novo to the patient.

875 4.5.2.1 Recurrent urine or fecal (flatal) incontinence: Observation of 876 involuntary, extra-urethral loss of urine and/or extra-anal loss of flatus/faeces 877 on examination NEW.

878 4.5.2.2 Recurrent fistula defect: observation of, within a clinical history context 879 of previous fistula repair (i) a period of transient complete fistula wound healing 880 followed by delayed complications of wound healing causing fistula breakdown 881 and fistula re-formation, or (ii) fistula recurring within the interval from successful 882 treatment to recurrence of fistula after which another fistula forms. NEW

883

884 4.6 WOMEN DEEMED INCURABLE (WDI) SIGNS

885 4.6.1. Definition: The fistula in this case is “beyond repair” and may have never undergone 886 treatment, but usually the symptom history is consistent with Chronic Fistula. Symptoms may 887 be consistent with persistent fistula but there may also be symptoms consistent with 888 recurrent fistula. There may be multiple attempts at repair and operations for persistent 889 incontinence. WDI signs are often the the most severe forms of fistula signs, be it treated or 890 untreated. NEW

891 4.6.2 Extra-urethral incontinence: Observation of urine leakage through channels other than 892 the urethral meatus, combined with: (i) observation of severe or total loss of the bladder, 893 and/or (ii) Observation of a urinary tract fistula that exceeds local capacity for successful 894 anatomic treatment. CHANGED

895 4.6.3 Extra-anal incontinence: Observation of fecal or flatal leakage through channels other 896 than the anal verge, combined with: (i) observation of severe or total loss of the anorectum, 897 and/or (ii) observation of an anorectal fistula that exceeds local capacity for successful 898 anatomic treatment. NEW

899 Footnotes for Section 4

900 FN 4.1 It is important to take into consideration past history during examination and 901 evaluation of the fistula e.g. radiation therapy. The signs will be documented according to 902 anatomic findings.

903 FN 4.2 Although the fistula is described from discrete anatomical sites, the fistula may involve 904 2 or more sites e.g. urethro-vesico-vaginal fistula. A ‘vault or cuff fistula’ is often a name given 905 to a post-hysterectomy fistula from the bladder to the vagina. A ‘cuff fistula’ is a vesico-vaginal 906 fistula.

907 FN 4.3 Urethro-vaginal fistula: There may be a common cavity made up of the anterior vaginal 908 wall with a defect at or above the level of the bladder neck, indicative of total loss of the 909 urethra (anterior and posterior walls) in the most extreme form – very difficult to cure. Lesser 910 urethra deficiencies may involve variable degrees of loss of the urethra distal to the bladder 911 neck, or congenital or acquired hypospadias.

912 FN 4.4 Uretero-colonic fistula - this may be iatrogenic after ureteric diversion into the bowel 913 for example, in the management of women with complex recurrent or persistent urinary 914 fistula symptoms.

915 FN 4.5 PFRD signs can result from: neuropraxia of the sacral nerve roots (which control lower 916 extremity function as well as bladder/bowel function), pelvic fibrosis, vaginal stenosis, cervical 917 atrophy or stenosis, diastasis or exposure of the pelvic bones.

918 FN 4.6 In such cases, it is common for intraoperative post-closure blue test to be negative, 919 with clear urine pooling in the fornix, indicating persistence of an upper urinary tract (ureteric) 920 fistula that may not have been diagnosed pre-surgery.

921 922 SECTION 5: INVESTIGATIONS

923

924 5.1 DYE AND BUBBLE TESTS FOR PFF:

925 Dye tests may be used to detect small or unusual fistulae (less useful for large or multiple fistulae), 926 such as utero-vaginal or cervico-vaginal fistulae and to differentiate ureteric fistula (clear or yellow 927 urine in vault, “negative dye test with urine in vault”) from bladder fistula (“positive dye test”) or to 928 detect small or distorted anorectal fistula (positive vaginal bubble or rectal dye test). Dye and bubble 929 tests are typically done at time of clinical examination for PFF, thus their inclusion in the “Signs” 930 section. NEW

931

932 5.1.1 Simple dye test for urinary tract fistula:

933 The bladder is filled retrograde through a urethral catheter using a dye to change the colour of the 934 irrigation fluid e.g. methylene blue or indigo carmine to turn the irrigation fluid blue (Figure 9). 935 Observation may begin with or without retractor(s) in the vagina, depending on digital and visual exam 936 signs and patient symptoms, or following careful dissection. Blue fluid leakage per genital tract or per 937 anus indicates a bladder or urethral fistula. Lack of blue fluid leakage combined with visualization of 938 extra-meatal clear urine leakage increases suspicion of an upper urinary tract ureteric fistula. NEW

939

940 5.1.2 Triple swab test for urinary tract fistula:

941 Three separate sponge swabs, one above the other, are placed in the upper, middle and lower vagina. 942 The bladder is then filled with a coloured irrigant such as diluted methylene blue, and the swabs are 943 removed after 10 minutes (it can take up to 30 minutes for urine to come through a tiny tortuous 944 fistula especially if it is in the cervix or uterus). Discolouration of only the lowest swab supports 945 diagnosis of a low urethral fistula or urethral leakage. Diagnosis of a uretero-genital fistula is 946 supported when the uppermost swab is wet but not discoloured. A vesico-genital fistula diagnosis is 947 supported when the upper swabs are wet with blue irrigant. Careful observation for backflow of blue 948 irrigant per meatus must be ongoing to avoid false-positive test reporting. NEW

949

950 5.1.3 Double dye test for urinary tract fistula:

951 This includes oral intake of phenazopyridine (pyridium) 200 mg three times a day for one to two days 952 until urine is bright orange, followed by retrograde bladder filling with blue irrigant through a bladder 953 catheter. Diagnosis of a bladder fistula to the genital tract is supported if the vaginal swab turns blue. 954 Diagnosis of a ureteric fistula to the genital tract is supported if the swab turns orange, combination 955 upper and lower urinary tract fistula to the genital tract is supported if the swab turns both blue and 956 orange. Careful observation for backflow of blue irrigant per meatus must be ongoing to avoid false- 957 positive test reporting. NEW

958

959 5.1.4 Trattner double balloon catheter test for urethral fistula:

960 The Trattner catheter has two balloons, one sits intravesically and the other inflates outside of the 961 meatus to block efflux from the urethra. The irrigant flows out through a lumen that sits between the 962 balloons, isolating fill to the urethra (Fig 9). NEW

963

964 5.1.5 Posterior wall irrigant/fluid per rectum for anorectal tract fistula

965 As with bladder dye testing, dye irrigation fluid may be instilled per rectal catheter. If coloured irrigant 966 passes per vagina, an anorectal fistula to the genital tract is confirmed. NEW

967

968 5.1.6 Posterior wall “bubble test” for anorectal tract fistula

969 With anterior vaginal wall retraction permitting visualization of the posterior vaginal wall, a Foley 970 catheter is inserted into the rectum, the balloon inflated, and held under traction against the anus. 971 Irrigant fluid is placed per vagina. A catheter-tipped, air-filled syringe is inserted into the catheter and 972 slowly decompressed to insert air into the rectum. Vaginal inspection allows visualisation of bubbles 973 emanating per vagina through a fistula defect. NEW

974

975 5.2. ENDOSCOPY EVALUATIONS FOR PFF AND PFRD:

976 These are normally not included in investigations in ICS documents, nor in the ICS Glossary. However, 977 they are an early assessment tool in the management of pelvic floor fistula and they generally precede 978 functional investigations.

979 5.2.1 Cystoscopy and ureteroscopy

980 Cystoscopy and urethroscopy may be used to better understand the configuration of upper and lower 981 urinary tract fistulas (Fig 13 A, B) and the proximity of the lower urinary tract to the ureteric orifice

982 FN5.1. NEW It will clearly identify other pathology, e.g. stone, tumor. Cystoscopy may, however, only be 983 possible in the smallest of fistulas where the bladder can still contain fluid (See Fig 13 A,B)

984

985 Figures 13: A: Cystoscopy in a fistula patient in Niamey, Niger. B Cystoscopic image of fistula defect. 986 ©L J Romanzi and Badlani.

987

988 5.2.2 Anoscopy and sigmoidoscopy

989 Lower gastrointestinal endoscopy may be used to better understand the configuration of upper and 990 lower anorectal tract fistula. Anorectal endoscopy is also helpful when evaluating PFRD of the

991 anorectal tract (Fig 14 A, B), such as stricture, residual anorectal incontinence, rectal pain syndromes 992 and compromised rectovaginal fistula wound healing. NEW

993

994 Figures 14: A: Anoscope; B Anoscope demonstration. ©L J Romanzi

995

996 5.2.3 Genital tract examination:

997 Vaginoscopy may be undertaken with any endoscopic equipment or nasal speculum. It is particularly 998 helpful in the evaluation of paediatric patients and women with severe vaginal stenosis. 999 Hysteroscopy make be undertaken to evaluate cervical patency and endometrial integrity for 1000 women reporting PFRD amenorrhea and/or infertility. NEW

1001

1002 5.3 BLADDER FUNCTION STUDIES FOR PFRD:

1003 There is no defined role for urodynamic investigations prior to the closure of urethral or bladder 1004 fistulas.

1005

1006 5.3.1 Functional evaluation (urodynamics) for Lower Urinary Tract PFRD 1

1007 5.3.1.1 Urodynamics (UDS): Measurement of all the physiological parameters relevant to the 1008 function and any dysfunction of the lower urinary tract40,41,

1009 5.3.1.2 Urodynamic usage in low resource regions: Multichannel urodynamics (MUDS) is 1010 becoming increasingly available in low resource regions A brief overview of urodynamics 1011 evaluation for common bladder pathologies occurring after fistula repair surgery will be 1012 reviewed in this document. Simple, single-channel urodynamics (“Simple Cystometrics”), a

1013 technique more commonly available in resource-constrained facilities, is also reviewed in this

1014 section FN5.2.

1015

1016 Figure 15: Simple UDS catheter placement and process overview.

1017

1018 5.3.2 Single channel urodynamics (“Simple Cystometrics”)42: Use of a catheter, catheter-tipped 1019 syringe and sterile irrigant solution, may provide rudimentary yet valuable information to guide 1020 treatment algorithms. Any residual fistula needs to be excluded. Simple ‘cystometrics’ requires the 1021 insertion of an indwelling catheter which is secured with inflation of the balloon (not present in Fig 1022 15) The bladder is filled with a catheter tipped syringe to approximately 300 mL of saline. The end of 1023 the catheter (after removing the syringe) is held vertically about 15 cm above the pubic symphysis and 1024 the level of the fluid in the catheter is noted. The volume for each filling sensation is noted. When 1025 there are no urge symptoms and no elevation of the meniscus, then the vesical pressure is considered 1026 ‘stable’. When the catheter is removed a cough test is performed to assess for stress urinary 1027 incontinence. NEW

1028

1029 5.3.3 Multichannel urodynamics40,41: Combines measurement of bladder and rectal pressures, filling 1030 volume and voided volume and urine flow rate (with or without video cystography). In centres where 1031 multi-channel urodynamics capacity exists, it is the preferred method for evaluating the complex 1032 aetiologies that often contribute to residual lower urinary tract dysfunction after fistula repair.

1033 5.3.3.1 Clinical sequence of urodynamics testing 1,2: Urodynamic investigations generally 1034 involve an individual attending with a comfortably full bladder for free (no catheter) 1035 uroflowmetry and post-void residual (PVR) measurement prior to filling cystometry and 1036 pressure-flow study.

1037

1038 5.3.4 Uroflowmetry:

1039 5.3.4.1 Ideal conditions for free (no catheter) uroflowmetry: Ideally, all free

1040 uroflowmetry studies should be performed in a completely private uroflowmetry room.

1041 Most modern uroflowmeters have a high degree of accuracy (+/- 5%) though regular

1042 calibration is important.

1043 5.3.4.2 Urine flow: Urethral passage of urine where the pattern of urine flow may be. 1,2,43,44:

1044 5.3.4.2.1 Continuous urine flow: no interruption to urine flow.

1045 5.3.4.2.2 Intermittent urine flow: urine flow is interrupted.

1046 5.3.4.3 Urine Flow rate (UFR – unit: mL/s): Volume of urine expelled via the urethra per unit 1047 time.1,2,43,44

1048 5.3.4.4 Voided volume (VV – unit: mL): Total volume of urine expelled via the urethra during a 1049 single void. 1,2,43,44

1050 5.3.4.5 Maximum (urine) flow rate (MUFR – unit: mL/s) – Qmax: Maximum measured value of the 1051 urine flow rate corrected for artefacts. 1,2,43,44.

1052 5.3.4.6 Flow time (FT – unit: s): Time over which measurable flow actually occurs. 1,2,43,44.

1053 5.3.4.7 Average (urine) flow rate (AUFR - unit: mL/s) – Qave: Voided volume divided by the flow 1054 time. 1,2,43,44.

1055

1056 Figure 16: A schematic representation of urine flow over time and parameters of uroflowmetry.

1057 5.3.4.8 Voiding time (VT – unit: s): Total duration of micturition, i.e. includes interruptions.

1058 When voiding is completed without interruption, voiding time is equal to flow time. 1,2,43,44.

1059 5.3.4.9 Time to maximum urine flow rate (tQmax – unit: s): Elapsed time from the onset of urine 1060 flow to maximum urine flow. 1,2,6,7.

1061 5.3.4.10 Interpretation of the normality of free uroflowmetry: Because of the strong dependency 1062 of urine flow rates in women on voided volume43 they are best referenced to nomograms43 where the 1063 cutoff for normality has been determined and validated and where the cut-off for abnormally slow 1064 (MUFR, AUFR) urine flow has been determined and validated as under the 10th centile of the 1065 respective Liverpool nomogram44.

1066

1067 Figure 17: Liverpool Nomogram for maximum urine flow rate in women43.

1068 Equation: Ln (Maximum urine flow rate) = 0.511 + 0.505 x Ln (voided volume)

1069 Root mean square error = 0.340 References: 22, 24 (Reproduced with permission)

1070

1071 5.3.5 Postvoid Residual (PVR) Volume of urine left in the bladder at the completion of micturition1,2,.

1072 5.3.5.1 Conditions for PVR measurement1,2: PVR reading is erroneously elevated by

1073 delayed measurement due to additional renal input (1- 14mls/min) into bladder

1074 volume. Ultrasonic techniques allow immediate (within 60 seconds of micturition)

1075 measurement45,46. A short plastic female catheter provides the most effective

1076 bladder drainage for PVR measurement.

1077 5.3.5.2 Assessment of normality of PVR: Quoted upper limits of normal may reflect

1078 the accuracy of measurement. Studies using “immediate” PVR measurement (e.g.

1079 ultrasound) suggest an upper limit of normal of 30mls. Studies using urethral

1080 catheterization (up to 10-minute delay) quote higher upper limits of normal of 50mL or more.

1081 An isolated finding of a raised PVR requires confirmation before being considered significant.

1082

1083 5.3.6 Filling Cystometry: is the pressure/volume relationship of the bladder during bladder filling 1084 1,2,6.7,2,40,41. It begins with the commencement of filling and ends when a ‘‘permission to void’’ is given. 1085 When multi-channel cystometry is done with fluoroscopy it is known as video cystometrogram or 1086 VCMG

1087 5.3.6.1: Cystometrogram (CMG): Graphical recording of the bladder pressure(s) and 1088 volume(s) over time. 1,2,6,7, 40,41,

1089 5.3.6.2 Conditions for cystometry including:

1090 5.3.6.2.1: Fluid: Water or saline unless radiological imaging.1,2

1091 5.3.6.2.2 Temperature of fluid: Fluid at room temperature is mostly used.1,2

1092 5.3.6.2.3 Position of patient: Sitting position is more provocative for abnormal 1093 detrusor activity (i.e. overactivity) than the supine position. 1,2

1094 5.3.6.2.4 Filling rate: A medium fill rate (50 mL/min) should be applicable in most 1095 routine studies. Much slower filling rates (under 25 mL/min) are appropriate in 1096 women in whom there are concerns about poor compliance (or with a bladder diary 1097 showing low bladder capacity or those with neuropathic bladder. 1,2

1098 5.3.6.3 Intravesical pressure (Pves – unit: cm H2O): The pressure within the bladder (as directly 1099 measured by the intravesical catheter)1,2,40,41

1100 5.3.6.4 Abdominal pressure (Pabd – unit: cm H2O): The pressure in the abdominal cavity 1101 surrounding the bladder. It is usually estimated by measuring the rectal pressure or vaginal

1102 pressure, though the pressure through a bowel stoma can be measured as an alternative. FN3.11

1103 The simultaneous measurement of abdominal pressure is essential for interpretation of the 1104 intravesical pressure trace1,40,41. Artifacts on the detrusor pressure trace may be produced by 1105 a rectal contraction1,40,41.

1106 5.3.6.5. Detrusor pressure (Pdet – unit: cm H2O): The component of intravesical pressure that 1107 is created by forces in the bladder wall (passive and active). It is calculated by subtracting

1,40,41 1108 abdominal pressure from intravesical pressure (P det = P ves – P abd) .

1109 5.3.6.6 Aims of filling cystometry: To assess bladder sensation, bladder capacity, detrusor 1110 activity and compliance as well as to document (the situation of and detrusor pressures 1111 during) urine leakage1.

1112 5.3.6.7 Bladder sensation during filling cystometry: Usually assessed by questioning the

1113 individual in relation to the fullness of the bladder during cystometry.

1114 5.3.6.7.1 First sensation of bladder filling: The feeling when the woman first becomes

1 1115 aware of bladder filling .

1116 5.3.6.7.2 First desire to void: The first feeling that the woman may wish to

1117 pass urine1.

1118 5.3.6.7.3 Normal desire to void: The feeling that leads the woman to want to

1119 pass urine at the next convenient moment, but voiding can be delayed if necessary1.

1120 5.3.6.7.4 Strong desire to void: The persistent desire to pass urine without the fear of 1121 leakage1.

1122 5.3.6.7.5 Urgency: Sudden, compelling desire to void which is difficult to

1123 defer1.

1124 5.3.6.7.6 Cystometric capacity: Bladder volume at the end of filling cystometry1.

1125

1126 Figure 18: 48 year old female with urinary frequency. No phasic activity during filling. Voided with 1127 normal urine flow rate at normal detrusor voiding pressure. Normal study. FD = First Desire to Void, 1128 ND = Normal desire to void, SD = Strong desire to void, U = Urgency, CC = Cystometric Capacity 1129 (permission to void given).

1130 5.3.6.8 Abnormal bladder sensation during filling cystometry

1131 5.3.6.8.1 Bladder oversensitivity1 –Increased bladder sensation during bladder filling 1132 with: (i) earlier first desire to void; (ii) earlier strong desire to void, which occurs at 1133 low bladder volume; (iii) lower maximum cystometric bladder capacity; (iv) no 1134 abnormal increases in detrusor pressure.

1135 5.3.6.8.2 Reduced bladder sensation: Bladder sensation perceived to be diminished 1136 during filling cystometry.

1137 5.3.6.8.3 Absent bladder sensation: No bladder sensation during filling cystometry, 1138 at least to expected capacity of 500mL.

1139

1140 5.3.6.9 Detrusor function during filling cystometry

1141 5.3.6.9.1 Normal detrusor activity/function2: There is little or no change in

1142

1143 detrusor pressure with filling. There are no detrusor contractions,

1144 spontaneous or provoked with activities such as postural changes, coughing or

1145 hearing the sound of running water.

1146

1147 Figure 19: 52 year old female with urgency and frequency. Phasic detrusor activity during filling. 1148 Leakage is associated with urgency and detrusor contractions. FD = First Desire to Void, ND = Normal 1149 desire to void, SD = Strong desire to void, U = Urgency, L = leakage, MCC = Maximum Cystometric 1150 Capacity.

1151

1152 5.3.6.9.2 Detrusor overactivity (DO)2: The occurrence of detrusor 1153 contraction(s) during filling cystometry. These contractions, which may be 1154 spontaneous or provoked, produce a wave form on the cystometrogram, of 1155 variable duration and amplitude. The contractions may be phasic or terminal. 1156 They may be suppressed by the patient, or uncontrollable. Symptoms, e.g. 1157 urgency and/or urgency incontinence or perception of the contraction may 1158 (note if present) or may not occur.

1159 5.3.6.9.2.1 Idiopathic (primary) detrusor overactivity2: No identifiable cause 1160 for involuntary detrusor contraction(s).

1161 5.3.6.9.2.2 Neurogenic (secondary) detrusor overactivity2,: Detrusor 1162 overactivity and evidence (history; visible or measurable deficit) of a relevant 1163 neurological disorder.

1164 5.3.6.9.2.3 Non-neurogenic (secondary) detrusor overactivity2: An 1165 identifiable possible non-neurological cause exists for involuntary detrusor 1166 contraction(s) during bladder filling. e.g. functional (obstruction); stone, 1167 tumor, UTI

1168 1169 5.3.6.10 Urethral Function During Filling Cystometry (Filling Urethro-Cystometry) 1170 Urethral closure mechanism 1171 5.3.6.10.1 Normal urethral closure mechanism2: A positive urethral closure 1172 pressure is maintained during bladder filling, even in the presence of increased 1173 abdominal pressure, although it may be overcome by detrusor overactivity. 1174 5.3.6.10.2 Incompetent urethral closure mechanism2: Leakage of urine occurs

1175 during activities which might raise intra-abdominal pressure in the absence of a

1176 detrusor contraction.

1177 5.3.6.10.3 Urodynamic stress incontinence (USI)2: Involuntary leakage of urine during 1178 filling cystometry, associated with increased intra-abdominal pressure, in the absence 1179 of a detrusor contraction.

1180 5.3.6.10.4 Subtype: Intrinsic sphincter deficiency (ISD)2: Very weakened urethral 1181 closure mechanism.

1182 5.3.7 Voiding cystometry1,2: (Pressure-flow studies): This is the pressure volume 1183 relationship of the bladder during micturition. It begins when the ‘‘permission to void’’ is 1184 given by the urodynamicist and ends when the woman considers her voiding has finished. 1185 Measurements to be recorded should be the intravesical, intra-abdominal, and detrusor 1186 pressures during the voiding urinary flow, including the urine flow rate. A partial synopsis 1187 of some voiding cystometry measures is included here. 1188 5.3.7.1 Pressure and other measurements during voiding cystometry:

1,2 1189 5.3.7.1.1 Detrusor opening pressure (unit: cm H2O) : Detrusor pressure 1190 recorded immediately before the commencement of urine flow.

1191 5.3.7.1.2 Flow delay (unit: s)2: The time elapsed from initial rise in 48

1192 pressure to the onset of flow. This is the initial isovolumetric contraction

1193 period of micturition. It reflects the time necessary for the fluid to pass

1194 from the point of pressure measurement to the uroflow transducer.

2 1195 5.3.7.1.3 Urethral opening pressure (Pdet-uo – unit: cm H2O) : Detrusor

1196 pressure recorded at the onset of measured flow (consider time delay – usually 1197 under 1 s).

2 1198 5.3.7.1.4 Maximum detrusor pressure (Pdet-max – unit: cm H20) : Maximum 1199 registered detrusor pressure during voiding.

2 1200 5.3.7.1.5 Detrusor pressure at maximum flow (Pdet-Qmax – unit: cm H2O) :

1201 Detrusor pressure recorded at maximum urinary flow rate.

2 1202 5.3.7.1.6 Detrusor pressure at end of flow (Pdet-ef – unit: cm H2O) :

1203 Detrusor pressure recorded at the end of urine flow.

1204 5.3.7.1.7 Postvoiding detrusor contraction2: An increase in detrusor

1205 pressure (Pdet) following the cessation of urinary flow (NEW)

1206 1207 5.3.7.2 Detrusor function during voiding cystometry 1208 5.3.7.2.1 Normal detrusor function: Normal voiding in women is achieved 1209 by an initial (voluntary) reduction in intra- urethral pressure (urethral 1210 relaxation). This is generally followed by a continuous detrusor contraction 1211 that leads to complete bladder emptying within a normal time span. Many 1212 women will void successfully (normal flow rate and no PVR) by urethral 1213 relaxation alone, without much of a rise in detrusor pressure. The amplitude 1214 of the detrusor contraction will tend to increase to cope with any degree of 1215 bladder outflow obstruction.

1216 5.3.7.2.2 Detrusor underactivity: Detrusor contraction of reduced strength

1217 and/or duration, resulting in prolonged bladder emptying and/or a failure to

1218 achieve complete bladder emptying within a normal time span.

1219

1220 1221 Figure 20: A schematic diagram of a pressure-flow study and pressure-flow parameters. 1222 1223 5.3.7.2.3. Acontractile detrusor: The detrusor cannot be observed to contract 1224 during urodynamic studies resulting in prolonged bladder emptying and/or a 1225 failure to achieve complete bladder emptying within a normal time span. The 1226 term ‘‘areflexia’’ has been used where there is a neurological cause but 1227 should be replaced by neurogenic acontractile detrusor 1228 5.3.7.2.4 Bladder outlet obstruction: This is the generic term for 1229 obstruction during voiding. It is a reduced urine flow rate and/or presence of a 1230 raised PVR and an increased detrusor pressure. It is usually diagnosed by 1231 studying the synchronous values of urine flow rate and detrusor pressure 1232 and any PVR measurements. A urethral stricture or obstruction due to higher 1233 degrees of uterovaginal prolapse or obstructed voiding after stress 1234 incontinence procedures are among possible causes. 1235 1236 Footnotes for Section 5 1237 FN 5.1 Cystoscopy may also be used to:

1238 • Evaluate suspected upper urinary tract fistula of the ureters through retrograde 1239 pyelography, to insert ureteric catheters at the time of repair of small lower urinary 1240 tract fistula that are in proximity to the ureters

1241 • To undertake ureteric catheter insertion for non-surgical treatment of ureteric fistula.

1242 • To evaluate persistent fistula-related disorders of the lower urinary tract, such as poor 1243 bladder compliance and reduced bladder capacity, foreign bodies, bladder and 1244 urethral diverticula, neurogenic bladder and drainpipe urethra.

1245 • Ureteroscopy may be used to diagnose ureteric fistula and to assess for PFRD co- 1246 morbidities of ureteric fibrosis and stenosis or ureteric stones through direct 1247 visualization. 1248 FN 5.2 Lower urinary tract symptoms (LUTS) may occur after closure of a lower urinary tract (bladder 1249 or urethra) fistula or may co-exist and persist after repair of an upper urinary tract (ureteric) or 1250 anorectal tract fistula. For persistent fistula-related disorders (PFRD) of the lower urinary tract, multi- 1251 channel urodynamics may be employed to evaluate complex bladder dysfunction symptoms that 1252 persist or occur de novo after successful PFF repair.

1253

1254 SECTION 6: IMAGING for PFF and PFRD

1255 This section profiles the imaging methods used worldwide in the evaluation of PFF and PFRD and 1256 defines the utility of each. Within the range of modalities, access and utilisation will vary depending 1257 on global location, level of health system capacity in each country, and level of facility within countries. 1258 Imaging methods and PFF/PFRD applications defined here are radiologic, ultrasound, magnetic 1259 resonance and computed tomography methods.

1260

1261 6.1 ULTRASOUND IMAGING

1262 6.1.1 Ultrasound 2-D methods. 1263 6.1.1.1.Transabdominal (T-A)1: curvilinear scanning applied to the abdomen.

1264 6.1.1.2 Perineal1: curved array probe applied to the perineum. Includes trans- 1265 perineal and trans-labial ultrasound. 1266 6.1.1.3 Introital1: sector probe applied to the vaginal introitus. 1267 6.1.1.4 Transvaginal (T-V)1: intravaginal curvilinear, linear array, or sector scanning.

1268 6.1.2 Ultrasound imaging 2-D PFF and PFRD applications 1269 6.1.2.1Bladder neck descent/mobility 1270 6.1.2.1.1 Urethral funneling: i.e. opening of the proximal third of the urethra during 1271 coughing or on Valsalva. NEW

1272 . 6.1.2.1.2 Urine loss: full urethral opening during coughing, Valsalva.

1273 bladder contraction or micturition. NEW

1274 6.1.2.2 Post void residual (PVR)1,2,45,46,47: See section 5.3.5 in investigations. 1275 6.1.2.3 Bladder and urethral masses/foreign bodies1: stone, tumour, foreign body 1276 or diverticula.

1277 6.1.2.4 Uterine, adnexal (upper genital tract) pathology1 – masses 1278 6.1.2.5 Pelvic organ prolapse1,3: Visualization of descent of the bladder, cervix/uterus 1279 and rectum during Valsalva and coughing 1280 6.1.2.6 Uterine version1,3: Anteverted, retroverted, flexion at isthmus 1281 6.1.2.7 Postoperative findings1,3,9: e.g. Bladder neck position and mobility, position of 1282 meshes, tapes or implants. 1283 6.1.2.8 Pelvic floor/levator ani muscle: voluntary control, defect (“avulsion”) and 1284 ballooning48,49. 1285 6.1.2.9 Bladder wall thickness, and ultrasound estimated bladder weight (UEBW).

1286 UEBW is higher in women with detrusor overactivity50.

1287

1288 6.1.3 Ultrasound imaging – 3-D methods

1289 6.1.3.1 Endo-vaginal ultrasound imaging may compress tissues, distorting the 1290 anatomy. 1291 6.1.3.2 Trans-anal ultrasound requires an expensive and dedicated transducer, is 1292 more uncomfortable and embarrassing. 1293 6.1.3.3 Trans-labial/trans-perineal minimizes tissue distortion and patient 1294 discomfort. 1295 1296 6.1.4 Ultrasound imaging 3-D PFRD applications

1297 6.1.4.1 Levator ani muscle (LAM): Trauma, atrophy, ballooning48,49. 1298 6.1.4.2 Anal ultrasound (Endosonography): This is the gold standard investigation in 1299 the assessment of anal sphincter integrity. There is a high incidence of 1300 defecatory symptoms in women with anal sphincter defects1,12.

1301 6.1.4.3 Urinary tract pathology: stones, scarring, diverticula, tumours or foreign 1302 bodies.1,2 1303 6.1.4.4 Other assessments: Synchronous ultrasound screening of the bladder and/or urethra 1304 and measurement of the bladder and abdominal pressure during filling and voiding 1305 cystometry.

1306

1307 6.2 RADIOLOGIC IMAGING

1308 6.2.1 Pyelography of the urinary tract: is a technique to generate an image of the upper 1309 and lower urinary tract by the introduction of radiopaque fluid (intravenous or retrograde

1310 via the ureter). FN 6.1

1,2 1311 6.2.1.1 Intravenous urography (IVU) : This provides an anatomical outline of the

1312 upper urinary tract, ureters and bladder as well as the evaluation of the kidney function 1313 and excretion of contrast media.

1314 6.2.1.2 Retrograde urethrocystography and voiding cystourethrography1,2: 1315 Unidirectional or combined contrast imaging of the urethra in a patient in the 30- 1316 degree oblique position to visualize the lumen mainly to diagnose urethral strictures or 1317 diverticulum. It is also of use to diagnose and stage urethral trauma.

1318 6.2.1.3 Retrograde pyelograms: may be performed when an IVU does not clearly define 1319 the anatomy of a suspected ureteral fistula.

1320 6.2.2 Video urodynamics 1,2 is a functional test of the lower urinary tract in which filling cystometry 1321 and pressure-flow studies are combined with real-time imaging of the lower urinary tract2 (Fig 22,23).

1322

1323 Figure 21 (left): Video urodynamics showing vesico-vaginal Fistula. © L J Romanzi and Badlani 1324 (L’Hopital Nacional de Reference, Niamey, Niger 2003)

1325 Figure 22 (right): Ureterovaginal fistula in a woman with a watery vaginal discharge. Video 1326 urodynamics was normal, but IVU shows obstruction of the left ureter (probably due to adjacent 1327 surgical clip (arrow), as well as a fistula, which is faintly outlined by contrast material and resultant 1328 opacification of the vagina. © L J Romanzi.

1329

1330 6.2.3 Hysterosalpingogram: is an imaging test to assess the endometrial cavity and fallopian tubes by 1331 introducing radiopaque fluid into the uterus. It may be used as an investigation for urinary and 1332 colorectal fistula tract into the uterus/cervix. NEW

1333

1334 6.2.4 Contrast enema: is used to identify colonic pathology12. It is a retrograde radio-opaque imaging 1335 technique that may assist in the diagnosis of an anorectal tract fistula. Due to the open anorectal tract 1336 preventing full luminal distension with radio-opaque contrast, a barium enema is prone to false 1337 negative images following subsequent evacuation12.

1338

1339 6.3 Computerized Tomography (CT)

1340 6.3.1 CT Urogram (CT-U)2: CT study of the urinary tract system using injected intravenous contrast, 1341 used to clarify diagnoses such as (i) tumors; (ii) renal disease; (iii) abnormal fluid collections/abscesses 1342 (iv) bladder pathology.

1343 6.3.2 CT Kidneys, ureter, bladder (CT- KUB)2: Non-contrast study aimed primarily at identifying stones 1344 but may identify other pathology. Also known as “stone protocol”.

1345 6.3.3 CT Imaging for fistula: Computed tomography (CT) role is limited for imaging fistula due 1346 irradiation load to the patient combine with poor CT resolution of soft tissue. Radiopaque contrast 1347 improves soft tissue resolution. However multi-planar spiral CT provides accurate visualization of the 1348 pelvic floor soft and bony structures by reconstruction of axial images using 1 mm thick slices without 1349 gaps that provides high pelvic floor diagnostic accuracy (i.e. LAM trauma or fistula tracts) (Fig 23) NEW

1350

1351 6.4 MAGNETIC RESONANCE IMAGING (MRI): In PFF, magnetic resonance imaging (MRI) maybe used 1352 to demonstrate concurrent conditions, such as urethral diverticulum and non-palpable abscesses. 1353 Though restricted in availability amongst low resource regions, where available, MRI imaging is helpful 1354 in cases of complex fistulae with adjacent organ system pathology.

1355

1356

1357 Figure 23: CT Urogram showing fistula between the bladder and the vaginal vault. © S Elneil

1358

1359 Footnotes for Section 6

1360 FN 6.1 Intravenous (antegrade) or retrograde pyelography may be used to evaluate for upper and 1361 lower urinary tract fistula, urethral diverticulum, tumours, strictures, stenosis, stones, foreign 1362 bodies, hydronephrosis, hydro-ureter and other upper and lower urinary tract disease, e.g., 1363 medullary sponge kidney.

1364

1365 SECTION 7: DIAGNOSIS

1366

1367 7.1 URINARY TRACT PFF DIAGNOSES:

1368 7.1.1. Definition: A diagnosis made by symptoms of a urinary tract fistula, signs of extraurethral 1369 leakage assisted by a probe or irrigant fluids (dye test), with imaging as required. NEW

1370 7.1.2 Genito-urinary tract fistula: an abnormal connection between the genital tract and urinary

1371 tract FN7.1. NEW

1372 7.1.2.1 Specific diagnoses for lower urinary tract may include: 1373 7.1.2.1.1 Deficiency of the urethra or urethrovaginal fistula (UVaF – 1374 See 2.1.1 and 4.3.2.1) -: Abnormal connection between the 1375 urethra and the vagina. NEW 1376 7.1.2.1.2 Vesicovaginal fistula (VVaF – See 2.1.2 and 4.3.2.2): 1377 Abnormal connection between the bladder and the vagina. 1378 NEW 1379 7.1.2.1.3 Vesico-vaginal-vault fistula (VVtF – See 2.3.3): Abnormal 1380 connection between the bladder and vaginal vault (cuff after 1381 hysterectomy). 1382 7.1.2.1.4 Vesico-cervical fistula (VCxF – See 2.4.1 and 4.3.2.3): 1383 Abnormal connection between the bladder and the cervix. 1384 NEW 1385 7.1.2.1.5 Vesico-uterine fistula (VUF – See 2.4.2 and 4.3.2.3): Abnormal 1386 connection between the bladder and the body of the uterus. 1387 NEW 1388 1389 7.1.2.2 Specific diagnoses for upper urinary tract may include: 1390 1391 7.1.2.2.1 Uretero-vaginal fistula (UrVaF – See 2.1.4): Abnormal 1392 connection of ureter into the vagina. NEW 1393 7.1.2.2.2 Uretero-cervical fistula (UrCxF – See 2.5.3): Abnormal 1394 connection of the ureter into the uterine cervix. NEW 1395 7.1.2.2.4 Uretero-uterine fistula (UrUF – See 2.5.3): Abnormal 1396 connection of the ureter into the body of the uterus. NEW 1397

1398 7.1.3 Colo-vesical fistula (CoVF – See 2.7.1 and 4.3.2.4): Abnormal connection between the bladder 1399 and either or both of the rectum and colon. NEW

1400

1401 7.1.4 Single or multiple fistula sites: The fistula may occur at a single or multiple sites with or without 1402 an ano/rectal/colo – fistula. NEW

1403

1404 7.2 ANORECTAL TRACT PFF DIAGNOSES

1405 7.2.1. Definition: A diagnosis made by symptoms of an anorectal, signs of extra-anal leakage of feces 1406 or flatus. assisted by a probe or irrigant fluids (dye test), with imaging as required. NEW

1407 7.2.2 Genito-anorectal fistula: an abnormal connection between the genital tract and the

1408 anorectum FN7.2. NEW

1409 7.2.3 Specific diagnoses: may include:

1410 7.2.3.1. Deficient perineum/total perineal defect: A spectrum of tissue loss from the perineal 1411 body and rectovaginal septum with variable appearance. There can be a common cavity made 1412 up of the anterior vagina and posterior rectal walls or just an extremely thin septum between 1413 the anorectum and vagina. NEW

1414 7.2.3.2 Fourth degree perineal tear (40PT): defined as an acquired childbirth injury and a 1415 subset of deficient perineum, involving both loss of the rectovaginal septum, full thickness 1416 anterior defect of the anal sphincter, and variable loss with lateral displacement of the 1417 fibromuscular architecture of the perineal body (cloacal-like defect). NEW

1418

1419 7.2.3.3 Rectovaginal fistula (RVaF- See 2.6.2 and 4.4.4): Abnormal connection between the 1420 rectum and the vagina.

1421 7.2.3.3 Recto-cervical fistula (RCxF -See 2.6.3): Abnormal connection between the 1422 rectum and the uterine cervix. NEW 1423 7.2.3.4 Recto-uterine fistula (RUF – See 2.6.3)): Abnormal connection between the 1424 rectum and the body of the uterus. NEW

1425 7.2.4.Complex recto-utero-cervical fistula

1426 7.2.4.1 Rectal/vaginal/perineal fistula (RVaPeF – Section 2.6.2.3) abnormal 1427 connection from the anal canal to the vagina or perineal area. NEW 1428 7.2.4.2 Recto-vesical fistula (RVF same as vesico-rectal fistula VRF – See 4.4.7 ): 1429 Abnormal connection between the bladder and the rectum. NEW

1430 7.2.4.3 Recto/colo-uterine/cervical fistula (RCoUF/RCoCxF See 4.4.5)): Defect 1431 between the colo/rectum and uterus (body and/or cervix). NEW

1432 7.2.5 Fistula in ano (FIA – See 2.6.4 and 4.4.8): An anal fistula is an abnormal connection between 1433 the anal canal

1434 epithelium (or rarely rectal epithelium) and the skin epithelium. CHANGED

1435 7.2.6 Single or multiple fistula sites: The fistula may occur at a single or multiple sites with or without 1436 a urinary tract fistula. NEW

1437

1438 7.3 INCONTINENCE DIAGNOSTIC CATEGORIES

1439 Fistula patients are typically pooled into three broad global health treatment outcome categories48. 1440 These are:

1441 7.3.1 Fistula closed and continent: fistula closed after treatment (surgical or non-surgical) without 1442 persistent or residual incontinence of the organ system (urinary tract or anorectal tract) that had the 1443 fistula. NEW

1444 7.3.2 Fistula closed and incontinent: fistula closed after treatment (surgical or non-surgical) with 1445 persistent or residual incontinence of the organ system (urinary tract or anorectal tract) that had the 1446 fistula. NEW

1447 7.3.3 Fistula not closed: fistula not closed during or after treatment (surgical or non-surgical). Not- 1448 closed fistula have defined subcategories including: NEW

1449 7.3.3.1 Persistent fistula diagnosis (See 3.4.1)– fistula that is not closed at conclusion of 1450 surgical or non-surgical intervention or that re-opens in the immediate post-intervention 1451 period. These treatment failures result from acute failure of wound healing or, in the specific 1452 case of failure to close the defect during surgical interventions, intra-operative failure of 1453 surgical technique. NEW

1454 7.3.3.2 Recurrent fistula diagnosis (See 3.4.2) – fistula that is closed post treatment, but 1455 recurs due to delayed failure of wound healing, or occurs subsequent to a follow-on index 1456 fistula-causing event. Subsequent index acquired fistula events are most commonly childbirth, 1457 surgery or pelvic trauma, but may also be inflammatory disease, infections and pelvic 1458 malignancy. NEW

1459

1460 7.4 WOMAN DEEMED INCURABLE (WDI)

1461 7.4.1 Woman deemed incurable (WDI) diagnosis (See 3.5.7): Women with primary, persistent and 1462 recurrent fistula for which anatomic repair is not possible. WDI require either supportive management 1463 and/or a diversion procedure, or they have a fistula complexity that exceeds the capacity(s) of the

1464 highest available surgical facilityFN7.4

1465

1466 7.5 PFRD FUNCTIONAL URINARY DIAGNOSES

2 1467 STORAGE DYSFUNCTION (SD) FN5.1 Those diagnoses related to abnormal changes in bladder 1468 sensation, detrusor pressure or bladder capacity during filling cystometry.

1469 Bladder Factor

1470 7.5.1 Bladder oversensitivity (BO – See 5.3.6.8.1)1,2

1471 7.5.1.1 Definition1,2: Bladder oversensitivity, a clinical diagnosis made by symptoms and urodynamic 1472 investigations is defined as: increased perceived bladder sensation during bladder filling with specific 1473 cystometric findings of: (i) early first desire to void; (ii) early strong desire to void, which occurs at low 1474 bladder volume ; (iii) low maximum cystometric bladder capacity; and (iv) no abnormal increases in 1475 detrusor pressure. Specific bladder volumes at which these findings occur vary in different 1476 populations.

1477 7.5.2 Detrusor Overactivity (DO – See 5.3.6.9.2)2

1478 7.5.2.1 Definition1,2: This diagnosis by symptoms and urodynamic investigations is made in 1479 individuals with lower urinary tract symptoms, more commonly overactive bladder symptoms 1480 when detrusor muscle contractions occur during filling cystometry.

1481 7.5.2.2 Subtypes

1482 (i) Idiopathic (primary) detrusor overactivity2 (See 5.3.6.9.2.1): no identifiable cause for the 1483 involuntary detrusor contraction(s).

1484 (ii) Neurogenic (secondary) detrusor overactivity2 (See 5.3.6.9.2.2): There is detrusor 1485 overactivity and evidence (history; visible or measurable deficit) of a relevant neurological 1486 cause.

1487 (iii) Non-neurogenic (secondary) detrusor overactivity2 (See 5.3.6.9.2.3): An identifiable 1488 possible non-neurological cause exists for involuntary detrusor contraction(s) during bladder 1489 filling. e.g. functional (obstruction); stone, tumor, UTI.

1490 7.5.3 Reduced compliance storage dysfunction (RCSD)2: This diagnosis by symptoms and urodynamic 1491 investigations is made in individuals with lower urinary tract symptoms, more commonly storage 1492 symptoms, when there is a non-phasic (at times linear or exponential) rise in detrusor pressure during 1493 filling cystometry with generally reduced capacity indicating reduced compliance.

1494 7.5.3.1 Reduced compliance (RCSD) incontinence2: urinary incontinence directly

1495 related to the RCSD.

1496 7.5.4 Outlet Factor (Urethra/Sphincter dysfunction - decreased urethral resistance – incompetence 1497 /insufficiency)

1498 7.5.4.1 Urodynamic stress incontinence (USI – See 5.3.6.10.3))1,2

1499 7.5.4.1.1 Definition1,2: This clinical diagnosis by symptom, sign and urodynamic investigations 1500 involves the finding of involuntary leakage during filling cystometry, associated with increased

1501 intra-abdominal pressure, in the absence of a detrusor muscle contraction.

1502 7.5.4.1.2 Subtype: Intrinsic sphincter deficiency (ISD – See 5.3.6.10.4)2: Very weakened 1503 urethral closure mechanism.

1504 VOIDING DYSFUNCTION (VD)2 Those diagnoses related to abnormally slow and/or incomplete bladder 1505 emptying manifest as an abnormally slow urine flow rate and/or an abnormally high post-void residual 1506 with confirmation by pressure-flow studies (including any related imaging).

1507 7.5.5 Bladder factor – (poor or absent detrusor activity)

1508 7.5.5.1 Detrusor underactivity (DUA – See 5.3.7.2.2))2

1509 7.5.5.1 Definition of DUA2: A diagnosis based on urodynamic investigations generally (but not 1510 always) with relevant symptoms and signs manifest by low detrusor pressure or short detrusor 1511 contraction in combination with a low urine flow rate resulting in prolonged bladder emptying 1512 and/or a failure to achieve complete bladder emptying within a normal time span, with or 1513 without a high postvoid residual (c.f. “hypocontractile detrusor” – detrusor contraction of 1514 reduced strength)

1515 7.5.5.2 Detrusor acontractility (DAC - See 5.3.7.2.3) 2

1516 7.5.5.2.1 Definition of DAC2: A diagnosis by urodynamic investigation, generally (but not 1517 always) with relevant symptoms and signs manifest by the absence of an observed detrusor 1518 contraction during voiding studies resulting in prolonged bladder emptying and/or a failure to

1519 achieve complete bladder emptying within a normal time span.

1520 7.5.5.2.2 Subtypes:

1521 − Neurogenic detrusor acontractility2

1522 − Non-neurogenic detrusor acontractility2

1523 7.5.6 Outlet factor (Urethral/ Sphincter dysfunction)

2 1524 7.5.6.1 Bladder outlet obstruction (BOO)

1525 7.5.6.1.1 Definition of BOO2: A diagnosis based on urodynamic investigations (pressure-flow 1526 studies +/- imaging), generally (but not always) with relevant symptoms and/or signs, manifest 1527 by an abnormally slow urine flow rate with evidence of abnormally high detrusor voiding 1528 pressures and abnormally slow urine flow during voiding cystometry with or without an 1529 abnormally high PVR.

1530 7.5.6.1.2 Possible sites/causes of BOO: Can be:

1531 5.4.1.2.1: Functional2: bladder neck obstruction, detrusor sphincter

1532 dysfunction, pelvic floor overactivity. (NEW)

1533 5.4.1.2.2 Mechanical2: urethral stricture, meatal stenosis). Video urodynamics can

1534 sometimes be required to ascertain the cause/site.

1535 7.5.7: Alternate presentations of voiding dysfunction

1536 7.5.7.1 Acute retention of urine2: An individual is unable pass any urine despite having a full 1537 bladder, which on examination is painfully distended, and readily palpable and/or percussible. 1538 (CHANGED)

1539 7.5.7.2 Chronic retention of urine2: Generally (but not always) painless and palpable or 1540 percussible bladder, where there is a chronic high PVR. The patient experiences slow flow and 1541 chronic incomplete bladder emptying but can be asymptomatic. Overflow incontinence can 1542 occur.

1543 7.5.7.3 Acute on chronic retention2: An individual with chronic retention goes into acute 1544 retention and is unable to void.

1545 7.5.7.4 Retention with overflow2: Involuntary loss of urine directly related to an excessively full 1546 bladder in retention.

1547

1548 7.6 PFRD – OTHER DIAGNOSES

1549 7.6.1 Pelvic organ prolapse1,3 (See 3.7)

1550 7.6.1.1 Definition: This diagnosis by symptoms and clinical examination, assisted by any 1551 relevant imaging, involves the identification of descent of one or more of the anterior vaginal 1552 wall (central, paravaginal or combination cystocele), posterior vaginal wall (rectocele), the 1553 uterus (cervix) or the apex of the vagina (vaginal vault or cuff scar) after hysterectomy. The 1554 presence of any such sign should correlate with relevant POP symptoms.

1555 7.6.2. Recurrent Urinary Tract Infections1 (See 3.6.3)

1556 7.6.2.1 Definition: This diagnosis by clinical history assisted by the results of diagnostic tests 1557 involves the determination of the occurrence of at least three symptomatic and medically 1558 diagnosed urinary tract infection (UTI) over the previous 12 months.

1559 7.6.3 Anorectal incontinence (See 3.10.10):

1560 7.6.3.1 Definition: This diagnosis is made by symptoms and clinical examination assisted by the 1561 results of investigations (anorectal manometry) and imaging (endoanal ultrasonography). At 1562 times, endoscopic evaluation may be required. NEW

1563 7.6.3.2 Sphincteric anorectal incontinence: Anal sphincter defects or weakness are present. 1564 NEW

1565 7.6.3.3 Urge anorectal incontinence: Incontinence is due to involuntary anorectal spasms. 1566 NEW

1567 7.6.3.4 Artefactual anorectal incontinence: Infective, inflammatory or neoplastic aetiology is 1568 identified. NEW

1569

1570 Footnotes for Section 7

1571 FN7.1 The diagnosis of urinary tract PFF may be defined by the anatomical location of the fistula (see 1572 Section 4) e.g. urethra-vaginal fistula. Larger fistulas often occur over more than one anatomical site 1573 e.g. involving both urethra and bladder.

1574 FN7.2 The diagnosis of anorectal tract PFF may be defined by the anatomical location of the fistula 1575 (see Section 4) but larger fistulas may occupy more that 1 anatomical site.

1576 FN7.3 The making of a WDI diagnosis is often, but not always, conditional. Of all categories, this is 1577 perhaps the most difficult diagnostic group and will be discussed further. Women in this category 1578 suffer with fistulas that are beyond the health system’s capacity to repair in an anatomically normal 1579 way, or who are unable or unwilling to undergo diversion of the urinary or anorectal tract for non- 1580 anatomic repair of their fistula.

1581 The categorization of women with fistula as “incurable” often occurs in the context of evaluation by a 1582 single clinician, usually but not always a fistula surgeon of variable level of expertise, working in an 1583 under-resourced environment with systems gaps that preclude achievement of a minimum acceptable

49 1584 standard of care for complex, elective reconstructive surgery .

1585 The limitations to single-surgeon diagnosis for WDI include49: (i) Informed only by their skills and 1586 experience; (ii) Criteria not standardized; (iii) Patient is often excluded from the decision process; (iv) 1587 Patient is often not adequately counselled on her health situation

1588

1589 FN 7.4 It could include those women who have their fistula closed but still remain incontinent despite 1590 repeated operations for ongoing incontinence)

1591

1592 FN 7.5 Limitations of WDI Diagnostic Criteria include but are not limited to:

1593 (i)Fistula complexity that precludes reconstruction of normal pelvic anatomy due to significant loss of 1594 tissue (bladder, anorectum, vagina) with or without dense pelvic fibrosis and/or vaginal stenosis; (ii) 1595 Socio-cultural and/or geopolitical constraints that preclude safe non-anatomic diversion and/or graft- 1596 based reconstructive surgery (bladder augmentation, intestinal or other graft source neo-vagina, etc); 1597 (iv) Health systems constraints that preclude successful service provision of advanced, complex 1598 anatomic or non-anatomic reconstructive surgery including staff (surgeon, anaesthetic, nursing), 1599 facilities/equipment/infrastructure, accessibility and affordability.

1600 63

1601 SECTION 8: CONSERVATIVE (NON-SURGICAL) MANAGEMENT51-53

1602

1603 8.1 Conservative management13: restricted to non-surgical and non-pharmacological treatments.

1604 8.2 Lifestyle interventions

1605 8.2.1 Indications: lifestyle intervention may be optimized to manage the chronic incontinence in:

1606 8.2.1.1: Non-surgical: Women who are not candidates for surgical treatment. NEW

1607 8.2.1.2: Surgery not preferred: Women who prefer not to undergo surgical treatment.

1608 NEW

1609 8.2.1.3: Urinary catheter not possible: Women who are also not candidates for non-

1610 surgical catheter treatment. NEW

1611 8.2.2 Types of lifestyle interventions (urinary incontinence): 1612 8.2.2.1: Adequate hydration: to reduce urine dermatitis on the vulva, legs and feet. NEW 1613 8.2.2.2: Skin protection: Protective dermal emollients for the vulva, legs and feet. NEW 1614 8.2.2.3: Pads: Adequate, preferably reusable, large pads or adult diapers13. 1615 8.2.2.4: Urethral plugs13,54: Products to block the urethral meatus in women with stress 1616 urinary incontinence after fistula closure. CHANGED.

1617 8.2.2.5 Vaginal lubricants13 : Pharmacological preparations aimed at reducing friction 1618 during coital or any other sexual activity and therefore alleviating dyspareunia, or at least 1619 reducing discomfort associated with clinical examination of the vagina or rectum. 1620 Pharmacological and natural plant-based oils may be used.

1621 8.2.2.6 UTI prophylaxis: Prophylactic antibiotics/antibacterial (e.g. methanamine) to 1622 reduce the incidence of recurrent or postcoital UTI. 1623

1624 8.2.3 Types of lifestyle interventions (anorectal incontinence) Anorectal lifestyle 1625 interventions include: 1626 8.2.3.1: Dietary modification: to minimize flatus and loose-liquid stool. NEW

1627 8.2.3.2: Skin protection: Protective dermal emollients for the vulva, legs and feet. NEW

1628 8.2.2.3: Pads: Adequate, preferably reusable, large pads or adult diapers13. 1629 8.2.3.4: Vaginal lubricants 13 : Pharmacological preparations aimed at reducing friction

1630 during coital or any other sexual activity and therefore alleviating dyspareunia, or at least 1631 reducing discomfort associated with clinical (per vagina or per rectum examination) 1632 Pharmacological and natural plant-based oils may be used.

1633

1634 8.3 Catheter insertion

1635 Inserting a catheter when an acute lower urinary tract injury is diagnosed may result in closure of the 1636 fistula, or reduced size of the fistula prior to subsequent surgical intervention 52.

1637 8.3.1 Bladder catheterization: may be used for secondary prevention or non-surgical treatment of 1638 bladder fistula55. NEW

1639 8.3.2 Ureteral catheterization (cystoscopic): may be used for secondary prevention or non-surgical 1640 treatment of ureteric fistula. Care must be taken to evaluate the healed ureter for secondary ureteric 1641 stenosis that may result in secondary obstructive nephropathy after fistula treatment. NEW Ureteric 1642 catheterization may be used during the repair of vesico-vaginal and ureteric fistulas. It is not a 1643 treatment for ureteric fistulas.

1644

1645 8.4 Physical therapy

1646 8.4.1 Pelvic physiotherapy - general: Assessment, prevention and/or treatment of pelvic floor 1647 dysfunction, performed by a pelvic physiotherapist. The therapy aims at reducing symptoms of fistula 1648 and post-fistula treatment incontinence symptoms as well as improvement of pelvic floor function56. 1649 The role of continence nurses amongst other health professionals in performing some of these 1650 specialized therapies is acknowledged.

1651

1652 8.4.2 Other therapies: covers many specialized therapies that can be used to train the pelvic floor 1653 including 13:

1654 8.4.2.1 Therapeutic exercise13: consists of interventions directed toward maximizing 1655 functional capabilities.

1656 8.4.2.2 Cognitive behavioural therapy 13: Cognitive techniques used in association 1657 with behaviour therapy principles. 1658 8.4.2.3 Bladder training 13: consists of a program of patient education, along with a 1659 scheduled voiding regimen with gradually adjusted voiding intervals. 1660 8.4.2.4 Bowel Habit training13: is aimed at establishing a regular, predictable pattern 1661 of bowel evacuation by patient teaching and adherence to a routine to achieve 1662 a controlled response to bowel urgency. 1663 8.4.2.5 Muscle training13: exercise to increase muscle strength, endurance, flexibility 1664 or relaxation. 1665 8.4.2.6 Coordination training13: is the ability to use different parts of the body 1666 together smoothly and efficiently. 1667 8.4.2.7 Biofeedback 13: is the use of an external sensor to give an indication with 1668 regard to bodily processes, usually with the purpose of changing the measured 1669 quality. 1670 8.4.2.8 Electrical muscle stimulation 13: is the use of electric potential or currents to 1671 elicit therapeutic responses. Current may be directed at motor or sensory functions. 1672

1673 In those fistula patients with flexure injuries, and/or foot drop, musculo-skeletal physiotherapy can be 1674 helpful in preparing the patient for surgery.

1675

1676 SECTION 9: SURGICAL MANAGEMENT

1677

1678 9.1 GENERAL FISTULA SURGICAL TERMINOLOGY

1679 9.1.1 Biological Grafts9: Any isolated healthy tissue or organ for transplantation into fistulous 1680 area to augment or strengthen the repair.

1681 9.1.1.1 Autologous grafts9: From patient’s own tissues e.g. rectus sheath or fascia lata.

1682 9.1.1.2 Allografts9: From post-mortem human tissue banks. Not often used in fistula 1683 surgery e.g. fascia lata.

1684 9.1.1.3 Xenografts9: From other species e.g. modified porcine dermis, porcine small 1685 intestine and bovine pericardium. Occasionally used in fistula surgery.

1686 9.1.2 Autologous grafts and flaps:

1687 9.1.2.1 Labia majora fat-flap : The use of labial fibro-adipose tissue underneath the labia

1688 majora FN9.1 (Fig 24) NEW

1689

1690

1692

1693 9.1.2.2 Labia minora flap: the use of labia minora to provide a skin flap to help reconstruct 1694 the vagina. NEW

1695 9.1.2.3 Buttock and perineal skin rotation flaps: the use of skin flaps from the 1696 buttock/perinea area to provide interposition fat and blood supply as well as increased 1697 vaginal skin surface area. NEW

1698 9.1.2.4 Peritoneal grafts and flaps: the use of peritoneum flap/graft to provide 1699 interposing tissue and blood supply as well as increased vaginal non-dermal surface area. 1700 It may be used at vaginal or abdominal surgery. NEW

1701 9.1.2.5 Omental flap: the use of omentum to provide interposing fat and blood supply 1702 during abdominal surgery. NEW

1703 9.1.2.6 Muscle flap: the use of muscle e.g. gracilis muscle or rectus abdominus muscle 1704 flap to provide tissue and blood supply. NEW

1705 9.1.2.7 Rectal advancement flap: mobilise/elevating a flap of the rectum above/below 1706 the fistula and using the flap to close over the fistula. NEW

1707 9.1.2.8 Singapore flap (pudendal thigh/groin vasculocutaneous crese flap): for vaginal 1708 reconstruction (not dissimilar to 9.1.2.3)

1709 9.1.2.8 Colonic flaps: for vaginal reconstruction of a large PFF in the presence of 1710 complete vaginal loss.

1711

1712 9.2 FISTULA REPAIR SURGERY

1713 9.2.1 Minor fistula surgery

1714 9.2.1.1 Cystoscopic cauterization of fistula: cauterisation of the fistula under direct vision 1715 via cystoscopy. Used for tiny fistula and may succeed. This is usually combined with 1716 prolonged catheter drainage. Theoretically, light (judicious) cautery destroys the fistula tract 1717 lining, allowing the bladder and vaginal tissues to heal (Fig 25). NEW

1718 9.2.1.2 Debridement of fistula: defined as removal of damaged tissue or foreign objects 1719 from a wound. May successfully be engaged as a primary therapy for small fresh 1720 rectovaginal fistula and adjunctively for non-surgical catheter treatment of vesicovaginal 1721 fistula 50. NEW

1722

1724

1725 9.2.2 Major Fistula Repair Surgery

1726 Principles of all fistula surgery include:

1727 9.2.2.1 Patient counselling: on the possibility of complications, including failure, and staged 1728 care. NEW

1729 9.2.2.2 Optimising patient health: operating on patients who are in optimal health for wound 1730 healing. NEW

1731 9.2.2.3 Tissue handling: careful tissue handling during dissection and suturing. NEW 1732 9.2.2.4: Wide dissection to well-mobilise the fistulised organs from each other. NEW 1733 9.2.2.5 : No tension: close the fistula defects under no tension. NEW 1734 9.2.2.6: Flaps and grafts: judicious use of autologous interposition flaps and grafts to 1735 assure adequate blood supply for wound healing. NEW 1736 9.2.2.7: Optimise functional result: attention paid to both form (close the hole) and 1737 function (restore normal function to the urinary, genital and anorectal tracts). NEW 1738 9.2.2.8 Intercurrent prolapse and incontinence surgery: Including but not limited to judicious 1739 use of prolapse reconstructive and incontinence procedures for concurrent pelvic floor 1740 disorders during the fistula repair. NEW

1741 9.2.2.9 Bladder drainage: catheterization

1742

1743

1744 9.3 MEASURING OUTCOME IN PELVIC FLOOR FISTULA SURGERIES11

1745 As per IUGA-ICS Report on outcome measures for pelvic floor surgery11, every study evaluating pelvic 1746 floor surgery should report.

1747 9.3.1 Perioperative data11: i.e. blood loss, operating time, length of hospital stay, return to 1748 normal activities and complications.

1749 9.3.2 Subjective (patient-reported) outcomes11: At its simplest level, this can be reported as the 1750 presence or absence of urinary/faecal incontinence. Patient satisfaction and quality of life can be 1751 measured by validated instruments that cover fistula, prolapse, urinary, bowel and sexual

1752 function. Also a consideration are reproductive outcomes e.g. menstruating, able to conceive and 1753 carry a pregnancy to term.

1754 9.3.3 Objective outcomes11: PFF-staging measurements tabulated with absolute values and 1755 percentages to allow other studies to compare results.

1756 9.3.4 Secondary outcomes11: (e.g. lower urinary tract symptoms, stress urinary incontinence or 1757 bowel and sexual dysfunction) in their studies whenever possible.

1758 9.3.5 Surgery type:

1759 9.3.5.1 Primary surgery11: indicates the first procedure required for treating PFF in any 1760 compartment.

1761 9.3.5.2 Further surgery11: provides a term for any subsequent procedure relating to 1762 primary surgery. Further surgery is subdivided into:

1763 9.3.5.2.1. Primary surgery in a different site/compartment.

1764 9.3.5.2.2 Repeat surgery in the same site/compartment for PFF symptom 1765 recurrence. 1766 9.3.5.2.3 Surgery for complications e.g. pain, infection, 1767 recurrent/persistent incontinence or haemorrhage. 1768 9.3.5.2.4 Surgery for non-PFF-related conditions usually prolapse, new 1769 onset urinary (e.g. stress urinary incontinence) or flatal/fecal 1770 incontinence. 1771

1772 9.3.6: Complications of PFF surgeries

1773 Complications related to PFF native tissue repair and surgeries using graft have been 1774 classified separately according to joint IUGA/ICS recommendation10. The system used in both 1775 documents utilizes specific category, time and site taxonomy together referred as CTS 1776 (Category, Time, Site) classification system.

1777

1778 Classification is aided by on line calculators at either http://www.ics.org/complication or 1779 http://www.ics.org/ntcomplication.

1780

1781 Footnotes for Section 9

1782 FN 9.1 The labia majora fat flap has blood supply both proximally (inferior epigastric and clitoral 1783 vessels) and distally (pudendal vessels). The flap may be divided at proximal or distal ends whilst 1784 maintaining its blood supply.

1785

1786 ACKNOWLEDGEMENTS / ADDENDUM:

1787 No discussion on terminology should fail to acknowledge the fine leadership shown by the ICS over 1788 many years. The legacy of that work by many dedicated clinicians and scientists is present in all the 1789 Reports by the different Standardization Committees. It is pleasing that the ICS leadership has 1790 generously supported this initiative as a means of progress in this important and most basic area of 1791 Pelvic Floor Fistula. On this project, we greatly welcome the collaboration with AUGS.

1792

1793 This document was initiated at ICS Tokyo (SE, BH – September 2016) and formalized in London (June 1794 2017 – SE Chair) with LR as Co-Chair (ICS Florence, September 2017) and JG as Co-Chair from early 1795 2019 with BH from October 2019. Working Group (WG) live meetings have been held in Florence 1796 (September 2017), Philadelphia (August 2018) and Gothenburg (September 2019). It has involved 16 1797 rounds of full review, by co-authors, of an initial draft (SE, LR). Formal editing, large sections of 1798 rewriting and additions as well as formatting then occurred (October 2019 – JG, BH with help from LR) 1799 to create Version 13 with a further 2 rounds of WG review. with the collation of comments (and Figures 1800 – Version 16). Following external review (8 experts – Version 17) and website publication (Version 18). 1801 Sign-off has included ICS Standardization Steering Committee (Version 19) and ICS Board. Version 20 1802 will be submitted for website and NAU journal publication.

1803

1804 We are extremely grateful the 8 expert external reviewers (Prof Ganesh Dangal, Dr Andrew Browning, 1805 Prof Dirk De Ridder, Dr Hannah Krause, Dr Chris Payne, Dr Tamsin Greenwell, Prof Sayeba Akhter, Dr 1806 Linda Ferrari). All of these colleagues provided excellent feedback. We thank the other colleagues who 1807 have provided comments on the website reviews (Name 1, Name 2…………………)

1808

1809 This document has been greatly enhanced by the medical illustrations of Levent Efe, 1810 www.leventefe.com.au, who’s ongoing work for ICS has been greatly appreciated

1811

1812 This document and all the NEW or CHANGED definitions will be uploaded to the ICS GLOSSARY 1813 (www.ics.org/glossary) where immediate electronic access to definitions and document download is 1814 available.

1815

1816 REFERENCES

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